Classification of tumors, etiology, pathogenesis, patterns of growth and development of benign tumors, principles of differential diagnosis and treatment. Benign tumors and tumor-like growths of sof t tissue MFA (papilloma, fibroma, lipoma, atheroma, dermoid and epidermal cysts, cyst neck medial and lateral, brahiogenic cysts and fistulas): etiology, pathogenesis, classification, histological structure, clinical features, differential diagnosis, treatment and prevention of complications.
Vascular tumors MFA, neurof ibromatosis.
1. General points, age and some clinical peculiarities, classification.
The unfavorable and worsening ecologic situation in
The mortality in oncologic diseases is higher, than from other diseases, what one may explain by great malignancy of their course, rapid growth and multiplication of tumors. Oral cancer is one of the few forms of cancer that has not experienced a significantly deceased mortality rate in the last 30 years. Deaths from oral cancer are higher than from cervical, testicular or thyroid cancer.
In the past 20 years, there has been an increasing interest in human papillomaviruses (HPV) because of their potential role in the pathogenesis of malignant tumors. In 1983, it was published the first evidence that HPV might be involved in oral squamous cell carcinomas. In a recent meta-analysis, HPV was indeed confirmed as an independent risk factor for oral carcinoma. To date, totally more than 100 types of HPV have been identified. As in anogenital cancers, HPV type 16 is the most prevalent type in oral carcinomas. The benign oral lesions, associated with HPV infection, include squamous cell papilloma, condyloma acuminatum, verrucca vulgaris and focal epithelial hyperplasia (FEH). Papillomas and condylomas are mostly caused by HPV type 6 or 11, while oral verrucas are associated with the skin types 2 or 4. A family history of FEH has been suggested. The FEH lesions are caused by HPV types 13 and 32, only detected in oral epithelium. In immunocompromised patients, benign HPV-induced lesions are characterized by atypical morphology and the simultaneous detection of multiple HPV types. Oral benign HPV lesions are mostly asymptomatic, and may persist or regress spontaneously.
We think it is purposeful to remind the row of some main points of oncology. By definitions of Abrykosov and Strukov, the tumor is the reactive growth of the tissue, which is different from physiological growth; namely, it is a pathologic manifestation of growth and development of tissue, and of cells, which is caused by malignancy of their flow, disturbance of physiologic mechanisms, regulating the development of tissues, their structure and function.
The real tumors have their peculiarities, which differ them from the similar by external signs, new formations (tumor-like) namely:
1. With removal of the reason of tumor formation, its growth is not stopped.
2. Pathologic peculiarities of tumor cells and pathologically changed metabolism of substances inside them –lead to the cell’s unlikeness and polymorphism and these signs are inherited by their ancestors. Many new peculiarities appear in them –the ability to germinate into other tissues and organs.
The pathologic signs of tumor growth are:
• atypicality of their multiplication – non-regulated and non-limited mitosis;
• atypicality of metabolism- in particular, oncoproteins’ synthesis;
•antigenic atypicality –which means the simplification of antigen composition;
•morphological atypicality –cell and tissue atypicity (cataplasia);
•functional atypicality;
•physico-chemical atypicity-in particular the saturation of tumor cells of hydrogen is increased, and this facilitates the diffusion of their substrates’ metabolism, and, along with this, the saturation by Calcium ions is decreased, what leads to worsening of inter- cell adhesion.
The benign or mature tumors consist of cells, by means of which one may determine, what tissue they originate from. They are called homological tumors.
The malignant or no mature tumors consist of slightly or no differentiated cells, which loose similarity to tissue, they have been formed from. The less the tissue is differentiated, the more malignant is its course.
Children make more than 15 % among patients with oral-facial area neoplasia. The frequency of malignant neoplasm of maxillo-facial area in children grew from 5 to 16% (L.M.Kliachkina) and it makes, according to recent data-12 cases per 100 thousand of children. Unfortunately, this growth is being continued. In averaged, the malignant tumors of head and neck make approximately 8% of all malignant tumors in child age (by data of oncologic institute in
The necessity of knowledge of oncologic aspects of pedodontics-is stipulated by causes, independent from age, and also stipulated by peculiarities of children’ organism development, namely:
1. The tumors of maxillo-facial area are located close to the brain and to main vessels and nerves of head and neck. The pointed out tumors may considerably worsen the process of food intake, breathing, eye functions, ear and nose functions, and also manifest visible cosmetic defects.
2. The age aspect means, that it is more difficult to determine in children, than in grown-ups, by
way of morphologic investigation, whether the cells are regarded to no mature, non-differentiated tissues, where these are the cells of malignant tumor, and that is why, in child’s age it almost impossible to establish exactly whether the tissue is benign or malignant (O.O.Kolesov,1991). This is also a case of fact, that 70% of children with malignant tumors of maxillo-facial area attend doctors with tumor processes, which have started much earlier, and now being in such degree, that is almost impossible to cure.
As
It’s necessary to mention, that a row of tumors in children, being congenital ones, may be combined with other defects, e.g. lymphangioma– with a row of defects, beginning from congenital cataract of eyes and tongue bifurcation, to congenital heart defects and lower extremities pathology.
Neoplasms of jaw bones are more often to happen in children 7-12 years old and it is connected with their increased growth, and more rarely –in children 12- 16 years old(endocrine re-formation), and very rarely –in children, aging 1 year. And concerning soft tissues of mouth cavity –the majority of diseases is observed at the age of 1, and in children 12-16 years old.
So, the tumor processes in small children are caused by disonthogenetic origin, but in 7-12 years old are caused by endocrine reformation and the abundant grows. Doctors observe the connection of children development acceleration with increase of specific weight of osteogenic sarcomas. When a person becomes older, the frequency of malignant tumors increases, what may be partly explained by larger durability of different oncogenic factors of influence (ecologic, harmful manners, chronic injuries, etc.).
According to growth character and clinics, all tumors are divided into benign and malignant ones.
Benign tumors grow slowly, as a rule, they are surrounded by capsule, and don’t grow, but only move apart the neighboring tissues or organs. They don’t form metastases and don’t relapse after radical removal. But keep in mind, that benign tumors may press on the neighboring organs, causing disturbance of their function, atrophia; they may tighten vessels and nerves, with different consequences. Sometimes they may threaten the life of a child, disturbing lively important functions.
The malignant tumors of MFA are most often diagnosed in children 3-4 and 7-10 years old, 80% of them are sarcomas. Malignant tumors grow rapidly and don’t have a capsule. They grow into other tissues and organs, ruining them. The cells of such tumors, growing into blood-carrying and lymphatic vessels, flowing with liquid stream, may be sometimes carried away to further located organs, causing the development of new tumors in them, called metastases. Such tumors cause disturbance of metabolism in the organism, its intoxication by products of life-activity, and at later stages of self decay-the self exhaustion (cohesion). Their infiltration growth hampers the determination of tumor borders. The infiltration growth leads to vessels destruction and bleedings, which sometimes may be mortal.
To make a correct diagnosis and for optimal organization of medical-prophylactic work concerning oncologic diseases of maxillo-facial area in children the doctors must know how to classify these diseases.
All tumors are classified into benign and malignant. They are divided into groups, depending on their origin, from the certain tissue, or from the certain organ. These groups take into account the known morphologic types of neoplasm.
BENIGN MALIGNANT
1. Epithelial tumors
2. Tumors of soft tissues.
3. Tumors of bones and cartilage.
4. Tumors of lymphoid and blood-forming tissue.
5. Tumors of mixed genesis.
6. Secondary tumors.
7. Tumors, which are not classified.
8. Tumor-like conditions.
As has been mentioned above, it is difficult to establish in children the distinct borders between benign and malignant tumors and that’s why such concept as neoplasm of transitional group –are applied (O.I.Paches, 1983).
At present, for theoretical and clinical application, the convenient classification of tumor processes in MFA in childhood is absent. The transfer of general oncologic schemes into pedodontics doesn’t give the needed result. Taking into consideration some remarks, one may accept the tumors’ division into benign and malignant ones, distribution according to histo-genesis and localization.
O.O. Kolesov classified tumors of jaw-facial area basing on clinico-morphological features, depicting 3 main groups:
1. Soft tissue tumors (55%)
2. Salivary glands’ tumors (5%)
3. Tumors and tumor-like neoplasm of jaw bones (40%).
The International histological classification of the head and neck tumors, accepted by IOHP, looks in the following way:
1. Skin tumors
2. Tumors and tumor-like neoplasm of the mouth cavity and pharynx, formed of many-layer flat epithelium
3. Salivary glands tumors and tumor-like neoplasm
4. Soft tissue tumors
5. Primary bone tumors and tumor-like neoplasm
6. Odontogenic tumors, tumor-like neoplasm and cysts of the jaw bones.
3 signs of anatomic spreading of neoplasm are put into the basis of International classification: spread of primary focus (T04-tumor); regional metastasing (N0x-bundle); remote metastasing (N0x-metastas).
According to E.Simanovska classification (1968), the benign tumors of soft tissues are divided into 3 main groups:
1. Tumors of mucous membrane and soft tissues:
• formed of epithelium (e.g. papiloma);
• formed of connective tissue and its derivatives (fibrome, hondrome, lipome);
• formed from muscle tissue (mioma);
• formed from nervous tissue (nervenome, neurofibrome, neurofibromatosis);
• formed from pigment tissue (pigment nevus);
• of vessel origin ( hemangioma, lymphangioma).
2. Salivary glands tumors:
• formed of epithelium (adenomas, mixed tumors);
• of connective tissue, vessels, nerves.
The tumor-like retention cysts are also included into this classification.
3. The tumors of skin and soft tissues under skin, and also tumor-like cysts –epidermoidal, dermoidal, atheromas.
The most often on children faces benign tumors of soft tissue appear. Further on, according to frequency, neoplasms of the bones appear. In the mouth cavity neoplasms of epithelial tissues prevail, the tumors of connective tissue origin-happen more rarely.
Basing on ICT principles, such benign tumors of soft tissues of mouth and pharynx are distinguished:-papiloma, fibroma, lipoma, rhabdomioma (from striped muscle tissue), hemangioma (capillary, cavernous, branchy, bunch-like, combined), lymphangioma( cavernous, branchy,combined), mixoma, myoblastoma.
Among neoplasms of skin and soft tissues of face in children-one may distinguish hemangiomas( capillary, venous, cavernous, cyst-like and arterial, venous, arterial-venous), lymphangiomas, neurofibromatosis.
Papilomatosis (nipple hyperplasia of mucous membrane), gum fibromatosis, giant-cell epulisis, dermoidal cyst, cysts of the salivary glands and ducts, adenomas-are related to tumor-like neoplasms. The epithelial tumors of salivary glands in children may be observed very rarely.
The benign tumors and tumor-like neoplasms of jaws are divided into:osteogenic, odontogenic and other origin. Such tumors as: osteoma, osteoblastoclastoma and tumor-like neoplasms: fibrous dysplasia, heruvism, hyperostosis, esinofilic granuloma, deformating ostosis– belong to osteogenic tumors. Among odontogenic tumors doctors may meet odontomas (hard and soft),adamantinomas, odontogenic fibromas; and from tumor-like neoplasm –cementoma, usual gigantic cell epulis and cysts-radicular, follicular, retromolar and primary odontogenic are distinguished.
The osteoma, chondroma, osteoblastoclastoma, desmoplastic and ossificated fibroma are related to benign bone tumors.
Among tumor-like neoplasms of jaw bones-one may distinguish their fibrous dysplasia and its special form-heruvism.
Fibrous Dysplasia is an uncommon benign fibro-osseous lesion disease, of unknown aetiology. It causes bone thinning and growths or lesions in one or more bones, and leads to bone weakness and scar formation within the bones.These lesions are tumor-like growths that consist of replacement of the medullary bone with fibrous tissue, causing the expansion and weakening of the areas of bone involved. The lesions can cause externally visible deformities.
Fibrous dysplasia usually occurs in children ages 3 to 15, but it sometimes is not diagnosed until adulthood. It is found equally between males and females.
Causes:
There are two types of fibrous dysplasia:
1. Monostotic (Involving a single bone, 70-80%),
2. Polyostotic (Involving many bones). The most severe form of polystotic fibrous dysplasia is known as Albright Syndrome. It was defined by the triad of polyostotic fibrous dysplasia of bone , skin pigmentation, and precocious puberty.
Symptoms may include
– bone pain (as a consequence of the expanding fibrous tissue in the bone)
– bone deformity
– bone fractures
Diagnostic procedures for fibrous dysplasia may include the following:
-x-ray
–biopsy
-computed tomography scan (Also called a CT or CAT scan). The radiograph shows the typical “ground glass” appearance replacing normal trabecular architecture.
– blood tests
Treatment for fibrous dysplasia:
may include:
- surgery, including the following:
- removal of affected bone, followed by bone grafting
- removal of bone wedge
- placement of a rod down the shaft of the bone
- medication
- pain management
- physical therapy.
Cherubism (familial fibrous dysplasia) is a rare genetic disorder (an autosomal dominant trait) characterized by abnormal bone tissue in the lower part of the face. Beginning in early childhood, both the mandible and the maxilla become enlarged as bone is replaced by fibrous tissue that is less dense, leading to swollen looking cheeks. Enlargement of the jaw usually continues throughout childhood and stabilizes during puberty.
The incidence of cherubism is unknown. At least 250 cases have been reported worldwide. It presents particularly in males, usually after the age of 4-5 years.
Mutations in the SH3BP2 gene have been identified in about 80 percent of people with cherubism.
Common clinical features of cherubism include:
- Bilateral swelling of the jaw (on both sides in the same area, although not necessarily to the same degree)
- More common in mandible than maxilla
- Upturned eyes (rim of sclera – the white of the eye – visible beneath the iris)
- Inverted V-shaped palate arches (the curved rear portion of the roof of the mouth)
- Enlarged submandibular lymphnodes
- Painless
- Premature loss of primary teeth
- Failure of permanent teeth to erupt or random distribution on eruption.
- Rapid development in childhood, slowing during puberty, later stabilization with possible regression.
- Sometimes, cherubism is associated with Noonan syndrome, which includes a typical facial appearance with low set ears, sunken chest, low platlets, generalized mild low bone density, and subtle (usually) heart malformations.
Diagnosis of cherubism is usually based on the above symptoms through a physical examination, family history, and imaging (panoramic X-rays and CT scans). Biopsies can be conducted to establish the presence of cell patterns typical of cherubism in the lesions.
Treatment options depend on the symptoms.
- Children with cherubism should be monitored by their physician and their dentist. If lesions are in the orbits an ophthalmologist should also monitor the optic nerve.
- If appearance causes serious image problems, surgery can be performed to remove the fibrous tissue. However, the potential for spontaneous regression of the disease suggests that surgical interventions should be delayed until after puberty.
- Malplaced teeth that hinder chewing and or cause serious image problems can be adjusted through orthodontia or extracted.
- Missing teeth can be replaced with prostheses.
- Radiation is NEVER recommended!
- To date, there is no established medical intervention into the growth phase although several scientific investigations have been conducted.
- Parents of affected childreeed to ensure that they have an opportunity to talk about their feelings about the condition, especially during the teen years.
- Parents should educate caregivers and teachers about the disease to decrease the possibility of teasing.
Fibro-osseous Dysplasia is a developmental, non familial, benign anomaly of bone development occurring in single or multiple bones, characterised by the replacement of normal bone by fibro-osseous tissue.
Eosinophilic granuloma ( Langerhans cell histiocytosis of bone) is a slowly-progressing benign tumor like condition characterized by an expanding proliferation of Langerhans cells in bones. Peak incidence is between the ages of 5 and 10 years and 75% of cases occur in persons under 20 years of age. Solitary painful bone lesions are the most common presentation, although they may be disseminated and polyostotic. Eosinophilic granuloma is one of the diseases that may have an associated sequestrum. The skull is usually involved, but lesions may be seen in any bone. Prognosis is excellent.
Etiology:
– occurs as a result of metabolic defects in the reticuloendothelia system;
– it is sometimes non painful, unless of course a fracture occurs;
– hallmark is presence of an osseous lesion (70-90%) most frequently arising in skull
– osseous lesions may produce rapidly destructive bone lesions.
Pathology:
Eosinophilic granuloma, which is classified with tumors of histiocytic origin, may be an isolated bony lesion or parenchymal (usually located in the cranium), or may be part of systemic disease (e.g. Letterer- Siwe disease or Hand-Schuller-Christian disease). Grossly, the tumor usually has ill-defined borders and a yellow-tan appearance. Microscopically, it is composed of large multinucleated giant cells with a polymorphous, eosinophil-rich inflammatory infiltrate. Special studies, including electron microscopy, may be necessary to resolve this differential. Electron microscopy shows distinctive structures called langerhans or Birbeck granules. These are rod like structures, with a striated core that may have a dilated end, giving them a tennis racket appearance
The differential diagnosis includes:
– Osteomyelitis:
– Ewing’s sarcoma: unlike EOG,
– Lymphoma
– Leukemia
– Hodgkin’s disease
– Myeloma:
– Intraosseous hemangioma
– Fibrous dysplasia.
Tests in children may also include:
- Biopsy of skin to check for the presence of Langerhans cells
- Bone marrow biopsy to check for the presence of Langerhans cells
- Complete blood count
- X-rays of all the bones in the body (skeletal survey)
Treatment:
– bone lesions often resolve spontaneously and do not require treatment unless they cause symptoms (less than 10% of lesions);
– curettage provides diagnostic biopsy material and is curative;
– with large lesions, bone grafts may be needed;
– injection of high dose steroids is another option and tends to result in rapid resolution of the lesion (often within 2 weeks);
– typical dose of steroid is 125 mg of methylprednisolone;
– chemotherapy or radiation therapy is usually not indicated
– XRT is indicated. The dose of XRT is usually between 500 to 1000 rads.
The pecularity of tumors and tumor-like neoplasm is that a row of them have odontogenic origin: adamantinoma (epithelial tumor; the structure of which is similar to the structure of enamel organ of dental germ); odontoma (soft and hard); cementoma, odontogenic fibroma, mixoma of jaw bones.
Cysts of jaw bones are divided into 3 groups: no epithelial, epithelial, and inflammatory. The simple and aneurismatic bone cysts belong to no epithelial cyst. The follicular, incisive and middle palatal cysts belong to epithelial ones. The inflammatory root cysts of jaws are also distinguished.
The tumors of jaw bones are frequently met in children 10-15 years old, and they are as of osteogenic origin, just so- of odontogenic origin (Paches, 1983). By the way, malignant tumors are observed to 10 times more rarely. And the most frequent of them is the odontogenic, the osteogenic and non-osteogenic sarcomas.
The neoplasms of salivary glands in children are met most frequently in large salivary glands, and very rarely –in small ones. In paired glands they, as a rule, are developed only on one side. The girls of 12-15 years old fall ill more often. The most frequent tumors of salivary glands in children are hemangioma, lymphangioma, and out of malignant tumors –no differentiated cancer and different sarcomas. At the same time when grown-ups have polymorphic adenomas around ear glands, children have prevailingly mucoepidermoid carcinomas and mixed tumors. In the sub-jaw gland in children- polymorphic adenomas develop prevailingly.
The clinic of large salivary glands in children is similar to that in grown-up persons. The main sign of benign tumors is a tight movable growth. The signs of malignant tumors are the mimic muscle paresis, when the tumor is localisated in the in the pre-ear gland; the trismus, metastases.
The sizes of neoplasms are observed in limits 2-5 cm. The anamnesis of the disease is, as a rule, rather short and it ofteotifies of the anti-inflammatory treatment of parotitis. It’s necessary to mention, that the most often mucodermoid carcinoma in children is less malignant, than in grown-up person.
The tumor injury of lymphatic nodes of the head and neck in children make 50-60% of all tumor pathology. There are lymphogranulematos, hematosarcomas and metastases. It’s not easy to diagnose the tumor of lymphatic system as the main part of children diseases is connected with the enlargement of the lymphatic nodes. The tumor injury of lymphatic nodes, especially on the initial stages, reminds the usual lymphadenitis. That’s why, in their durable enlargement- the doctor must carry out a cytological investigation. The correct differential diagnostics with leucosis depends on the detailed hematologic observation.
The skin tumors in children are rather frequent and have prevailingly a benign character. The epithelial malignant tumors in children, comparing to grown-up persons, are rare. Among benign tumors most often there are the vessel neoplasms, which in 2/3 of cases are localisated on the haired part of the head. They are revealed in 90-95% of newborn children. When they have been revealed in children, after some months of birth, it’s important to find out whether they are congenital. From the moment of their appearance one must observe, because in 60-70% hemangiomas become smaller or disappear when a child is 1 year old, especially on haired part of head. But it’s good to remember, that some increase of hemangioma may be observed before this. At the same time, the tumor may give a sudden very rapid growth and proliferate into the mouth cavity, nose or pharynx and it may lead to complex of functional disturbances.
Among unfrequented malignant tumors of skin in children melanomas prevail. The benign nevus is not, as a rule, dangerous, concerning melanomas.
Other malignant tumors of skin and soft tissues (sarcomas of different origin) are very rear and very aggressive. Also neuroblastomas are very malignant and unfortunately their first signs are metastases into the bones, liver, lungs. That’s why when there is a suspicion of neuroblastoma, the doctor must carry out a roentgenologic investigation of bones and lungs, USD of liver, and also, as L. Durnov recommended (1979), to study the cateholamines’ metabolism, which has the tendency to change.
Among malignant tumors of maxillo-facial area in children there are:
• osteogenic sarcoma
• fibrosarcoma
•
• reticular sarcoma
• lymphogranulomatosis
• lymphosarcoma.
The doctors also observe the cancerogenic injuries of different localization: of lips, cheeks, tongue, jaw bones. The malignant tumors in children are prevailingly primary tumors of connective-tissue origin: fibrosarcoma, angiosarcoma and reticulosarcoma.
Prior to tumor are tumor-like neoplasms, dysplasias, hyperplasias. For example, in development of malignant epithelial tumor-cancer, a considerable role plays the disturbance of mutual relation between epithelium and the connective-tissue, lying under it, as the epithelium growth is determined by structural-functional condition of this connective tissue. In case of pathology, the epithelium grows into it (germinates) and this is the first step to the tumor development.
This concerns tissue changes. And what is about cells? Where do the malignant tumors come from in organism? Each of them begins from 1 cell, which looses the mechanism of its growth regulation. Throughout a man’s life, many healthy cells are reborn in such way, but the tumor doesn’t appear in each of them. The cancerogenic factors favor it. And then from 1 typical cell the other similar cells develop. The development of many cells from 1 cell is called cloning. So, the tumor is a clone. It’s necessary to note that in a person only one tumor may develop, but not several ones, as in other diseases.
The majority of atypical cells, appearing in the organism, may die rapidly, because of limited blood supply, and a low metabolism level. The accumulation of milk acid stimulates the germination of vessels and mitosis of atypical cells. If more than 600 thousand of such cells have been formed, than the intensive growth of capillaries in them may happen and then this new foundation becomes a clinical tumor.
The group of pre-cancer conditions of face skin, red edges of lips and mucous mouth membrane is worth of attention, because their diagnostics and treatment are first of all the prevention of cancer and its discovery on early stages.
Pre-cancers are unstable proliferates, which have not yet become tumors. Their development may be reverse and they may be treated. Durable inflammatory processes, chronic traumas, tumor-like neoplasm and benign tumors may be named as pre-tumor conditions.
Doctors may distinguish a concept “pre-cancer” in its wide meaning. They mean all benign neoplasms, chronic inflammations, hormonal disturbances. And in a narrow meaning- the pre-cancer- is characterized by presence of specific morphologic changes in the epithelium, which may appear under the influence of chronic trauma, e.g. harmful habits. Here may happen the hyper- or diskeratosis of epithelium. According to Mashkileison classification, there are 2 main forms of cancer: obligate and facultative. The obligate pre-cancer is transformed into cancer and the facultative one-never. The latent period is only in obligant pre-cancers- it may last to 30-40 years.
The pre-cancer conditions of face skin are: obligant –Boyen disease and pigment xeroderm; facultative- radiational dermatosis, melanosis, keratosis of mucous mouth cavity-erythroplakia (obligant form), leucoplakia, red vovchak, flat lichen (facultative form); and also pre-cancer of red edge of lips as the facultative (on mucous membrane), just so- the obligant one- a wart-like pre-cancer, Manghanotti cheilitis, hyperkeratosis. Besides the pathologic conditions the so-called pre-cancer background diseases exist: chronic ulcers, cracks, scars after burns.
So, the neoplasm in jaw-facial area in children are characterized by a special clinical course and morphology, which must be taken into account in diagnostics and treatment, and be tightly connected with questions of organization of oncologic help, the most important of which is the early reveal of tumors and their timely treatment.
2. Diagnostics and organization of treatment of children with tumors of maxillo-facial area.
The comparatively high illness of children with tumor processes of tooth-jaw area demands the special attention concerning questions of organization of help to this group of patients. This is stipulated by low affectivity and not enough prophylactics of neoplasm, because their etiology and pathogenesis are not completely studied. That’s why only a timely diagnostics, in cases with malignant tumors – allows curing the patient and saving his /her life. It’s a pity, that the treatment of neoplasm, especially of malignant nature, and old ones – is not quite effective. The matter is, that in case of too late reveal and untimely treatment of benign tumors – the large sizes of post-operational defects and disturbance of a row of functions may be quite negatively marked on health and life activity of a sick child.
The success in achievement of positive result in oncologic patients depends first of all on the primary diagnosis, put by the first doctor. That’s why the organizational basis of oncologic help must be known by every doctor, because in this very case– the district dentists decide the question of early diagnostics of the disease.
In the basis of giving aid to children with oncologic dental pathology is the dispensary method of initiative character. It allows timely revealing and recognizing the true disease
during planned professional examinations, to give a timely specialized help, and also to reveal as early as possible the recurrences and metastases.
The children are not habitual to complain of pain, even in cases of large tumor sizes. At the same time, the grown-ups may notice some changes in the child’s behavior (irritability, weakness, tiredness, loss of appetite, disturbance of sleep).
The first clinical symptoms of tumors most often is a jaw deformation , which is revealed in asymmetry of face, irregular form of a tooth row, displacement of certain teeth, thickening of alveolar sprout, difficulty during opening the mouth or chewing, in swallowing. Some of teeth
may be movable or get loosen. The skin above a large tumor, as a rule, is not changed, and later on – it may be strained, revealing the picture of vessels. In presence of a malignant process, the tumor grows into skin layers and it is connected with them. The metastases on early stages are quite rare. The rapidity of tumor growth depends on its sizes – the larger the tumor, the more rapid is its growth.
Ieoplasm diagnostics – great role plays the study of life anamnesis, including the antenatal period, and the anamnesis of disease, the questions about the earliest symptoms of the disease. It could be complains on the swell of the face, neck, protrusion of the tongue, difficulty during chewing food, swallowing or pronunciation of words, change of a bite or position of separate teeth, defects of a bite or position of separate teeth, their movement, bleeding, appearance of ulcers, which don’t heal for a long time (during 2-3 weeks or more). The doctor must remember about the possibility of tumors development, in presence of some congenital defects.
During examination a doctor may reveal changes of skin color, or the color of mucosa, changes in the face configuration, swelling, incorrect position of some teeth, defects of occlusion (bite), mobility of teeth, thickening of alveolar sprout. Depending on the tumor localization, the doctor may guess about the type of a tumor.
The defects in correct placement of teeth – may be observed as in soft tissues tumors, just so in jaw bones tumors. (hemangiomas, fibromas, reticular sarcomas). The salivary glands tumors may occur as in the thickness of large salivary glands, just so– on the edge of hard and soft palates. Fibromas and papilomas may occur on the mucosal cover of cheeks, along the line of teeth occlusion. Lipomas are in thickness of cheeks; the retention cysts, hemangiomas, papilomas may occur on the mucus of lips. The fibro- and hemangiosarcomas may develop in the tongue thickness, and rhabdomiomas – may appear in muscles of sides of the tongue. Fibromas and fibroadenomas may occur near the tongue root. In babies – mioblastoma may appear under the gums cover.
Face configuration may change in case of hemangiomas, lymphangiomas, neurofibromatosis, cysts of large salivary glands. The limitation of jaw mobility, pain during opening of mouth –may be the sign of neoplasm localization in the wing-jaw space and in the pre-ear region.
After the examination of patient the doctor passes to the next stage of observation of the sick child, called palpation. By means of this method a doctor determines the character of the new-formation surface, its borders and consistency, mobility, relation to surrounding tissues, zone and depth of infiltration, painfulness. The palpation of soft tissues of jaw facial area is carried out bi-manually.
The reveal of pulsation in the tumor sickness – testifies to its good blood supply, saturation with vessels. The soft, dough-like thickness of the new formation, fluctuation testify to its nonosteogenic origin, presence of cyst or a cyst-like new formation, or the inflammatory process.
During palpation of lymphatic nodes doctors pay attention to the degree of their enlargement, their mobility, painfulness, form, consistency and connection with surrounding tissues. The enlarged, painless, combined into packets lymphatic nodes allow to make an assumption about the presence of metastases of malignant tumors.
The hyperplasias of neck lymphatic nodes, which may often occur in children, make it difficult to diagnose their tumors and metastases.That’s why, doctors must remember, that in case of hyperplasia, the nodes have the egg-like form, elastic consistency and their size is not larger than 1,5 cm. The characteristic is also the rapid (during 1-3 days) enlargement of nodes, pain in palpation, presence of a reason-“gates of infection”. In favor of neoplasm or metastasis is the presence of at least 1 painless of a round form- node, more than 1 cm in diameter, on the background of a general malaise, paleness, subfebrility, hemorrhagias.
Basing on the above mentioned data, received in the processs of collecting the anamnesis, complaints and clinical examination-the prepreliminary diagnosis is being put. To put a final diagnosis, a dentist must carry out the additional examinations: rentgenologic, including a computer tomography, radioisotopic scanning. The dentists often make a dento– and heiloscopy with a pre-life painting of tissues, and the angiography..The improvement of cytodiagnostics allows to reveal the cancer of mucosal layer at the pre-invasive stage.The aspirational or punctional biopsy according to d-r. Panikarovskyi method, helps to reveal the tumors located more deeply. But the most important in making a final diagnosis of the tumour is the pathomorphological investigation of material, received by method of invasive or punctional biopsy. But in children the enough informational pathomorphological investigation may manifest
a disputable pathomorphologic diagnosis,that may be explained by a disposition of a child to different tumour-like hyperplasiae, large similarity of nonmature tissues at early age of a child – to blastomatic ones, and also by specific for children morphologic similarity of a row of benign and malignant tumors.
Rather complex for children is a differential diagnostics of malignant and benign tumors, because the latter don’t have the specific symptoms at early stages of the disease. And
for benign tumors the more rapid growth is characteristic. It is rather difficult to differ a malignant tumor from a chronic osteomyelitis, because in children the latter happens more often
with an abundant bone formation and a rapidly progressing bone deformation.
In children, in connection with a porous bone structure, the destructive process may spread more rapidly, than in adults, and a rather high reparational potential of periostium assists to appearance of distinct signs of periostal bone formation on X-ray picture.
The evaluation of age and a general child’s condition, comparing and analysis of complaints, anamnesis and the results of clinical and paraclinical examination allow the doctor
to put a final diagnosis.
The reasons of late diagnostics are: weak clinical signs, especially at the early stages of tumorous process, incomplete knowledge of the early signs of tumors, self-treatment, too late visit to a dentist, many-stage examination (long lasting), diagnostic mistakes, non-grounded treatment. The tragedy of situation with oncologic diseases of jaw-facial area in children is stipulated by 3 reasons: the absence of oncologic caution of dentists-pediatritians, insufficient knowledge by dentists of early clinical manifestations of tumors, non ability to read the X-ray pictures, by psycho-physiological peculiarities of children – their non ability to tell the doctor in a true way their own feelings at early stages of the disease.
The tumors’ prophylaxis includes the antenatal protection of a fetus, maximally early reveal and removal of pre-tumor and the background processes, formation of dispensary groups of children with an increased risk of tumor appearance, in the hardened oncoanamnesis in mothers, who were irradiated or used cytostatics during pregnancy.
The good knowledge of oncology by dentists, knowledge of early signs of tumor
processes and main points of organization of medico–prophylactic work allows to ensure the optimal level of rendering a specialized help to children with oncologic diseases of maxillo-facial area.
The ideal opportunity for early detection of cancerous or pre-cancerous growths is during the annual hygiene examination. Unfortunately, the cellular changes that lead to oral cancer start below the surface of the epithelium at the basement membrane, making them difficult to detect by conventional screening methods.
The annual hygiene exam should basically comprise of traditional incandescent light examinations to enhance the visualization of oral mucosal abnormalities that may not be visible to the naked eye, such as oral cancer or pre-malignant dysplasia. There have been scientific breakthroughs in the world of dentistry giving them more accurate devices to detect oral cancer. The devices are portable and can be used by hygienists or dentists themselves and the examination takes only one or two minutes. The oral cancer detection devices use fluorescence visualization technology resulting in abnormal tissue appearing irregular, dark areas that stand out against the otherwise normal, green fluorescence pattern of surrounding healthy tissue.This significant advancement in early detection is very easy for the dental staff to administer and completely non-invasive for the patient. It takes very little time, is pain-free, and involves no distasteful rinses or staining.
The tests that may be used include:
· A X-ray,
- a magnetic resonance imaging scan,
- a computerized tomography (CT) scan, and
- a positron emission tomography (PET) scan.
A PET scan involves injecting a part of your body with a radioactive ‘tracer’ chemical which can be seen on a special camera. Further biopsies on nearby lymph nodes may also be carried out. The basis of early diagnostics of tumors of jaw-facial area in children is the oncologic caution of children dentists. In pediatric practice the principles of oncologic caution are formed in the following way:
1. Every doctor –dentist must know the symptomatic and clinics of main types of tumors
in children.
2. During examination of every patient by dentist, independently of the child’s age, complaints, clinics of main dental disease, it is necessary to exclude the presence of a tumor or pre-tumor condition in jaw-facial area, that’s why he (she) must be examined taking into consideration the possibility to reveal this pathology.
3. The basis of suspicion of oncologic pathology in patient is:
a) appearance of exofite formation with infiltrated basis, which is being enlarged in sizes, and bleeding;
b) presence of bleeding ulcer with an infiltrate in the basis,
c) appearance of constant pains of moderate intensivity in pathologic process zone, which are strengthened at night;
d) appearance of mobility of I or several intact teeth, accompanied by pain;
e) changes of character of nose secretions, in patients with chronic antritis (appearance of purulent secretions).
f) paresis of mimic muscles, which grows gradually, parastesias and numbling in zone of innervations by an “under eye” and under chin nerves.
g) the presence of circle-like tough painless or slightly painful (in palpation) lymphatic nodes on the neck, which may grow.
4. The doctor must observe the skin of neck and head of every patient, and in case of revealing any changes on it, to direct a child for examination of a dermatologist or oncologist.
5. The tumor process must be excluded in any unusual course of a disease or if it’s
impossible to explain some symptoms or if a clinical picture is not clear.
6. In case of atypical course of any disease, the paraclinical investigations must be carried out (hysto– and cytological, roengenological, laboratory), the doctor must consult the colleagues, to direct patients for consultations to specialists-oncologists.
7. The doctor must remember that any chronic or acute disease may be accompanied by oncologic pathology and press by its symptoms the signs of it. That’s why in rendering help in case of trauma or acute inflammatory process the oncologic caution must be manifested.
8. The examination of a child with a suspicion of tumor the doctor must carry out in short terms, by principle of urgent surgery.
9. All patients with malignant tumors must be discussed, so as to reveal the possible mistakes in prophylactic, diagnostic and medical work of doctors, in forming an oncologic caution in them.
10. The knowledge of doctors of organizational bases of oncologic help to children ensures the timely and correct direction of them by allocation for giving aid.
Here are some clinical cases, which illustrate the pointed out postulates.
A child, aged 15 months old, during a game with stick, which she kept in mouth, injured a mucous cavity of hard palate, in consequence of which a bleeding appeared, which stopped after some time without calling a doctor. After some time has passed, the parents noticed that an under mucosal bleeding on hard palate doesn’t disappear and they addressed the doctors with complains of this. During the examination a hemangioma of hard palate was revealed, may be a congenital one, which has not been revealed earlier. And only in connection with the trauma which a child has got, doctors by chance, diagnosed a tumor.
Here is another case. During pre-new year party, the parents of 5 year old boy addressed the surgeon on duty. The boy complained of general malaise, fatigue, increased body temperature, swelling and pain in the area of left corner of mandible, difficulty in opening the mouth. These symptoms lasted for 2 days. Basing on anamnesis, the doctor found out that during 10 months the boy has been treated by dentist in the case of chronic periodontitis of 75 tooth. As the preliminary extraction of this tooth may lead to irregular growth of teeth of permanent bite (such was the explanation of the doctor). The durable treatment of the tooth was complicated by periodical acuteness of the disease. The physic-therapeutic procedures have been prescribed. 4 month ago the doctors noticed the thickening of child’s jaw, where the tooth has been located. Rentgenologically hyperostosis has been revealed, the reasons of which was systematic acuteness of inflammatory process in the periodontium of treated tooth.
At that moment the child had all signs of acute inflammatory process of a bone in the area of left corner of mandible, regional lymphadenitis, trism of 3rd degree, body temperature was 38.2ºC. After the anesthesia has been carried out and trism removed it was revealed that 75th tooth has been ruined on 2/3 by caries, hyperemia, odema (swelling) of the surrounding mucosal cover, smoothing of transitional fold. The tooth was movable (3rd degree). After its extraction 3 ml of pus were excreted from the socket. The adequate medicament therapy has been prescribed.
In 2 days after repeated examination the child’s condition was much better. The body temperature lowered to subfebrile, the swelling in the injured area decreased and no signs of inflammation was observed. The painless tight thickening of a bone in the mandible corner of 2 cm in diameter was palpated. The regional lymphatic nodes were enlarged and painless. The socket was partially filled with a cluster, with greish coating, and its walls were of cartilage-like consistence of white color. The child was made a roentgenogram of mandible from the left side.
And in its corner area – a tumor was revealed with a characteristic of osteosarcoma structure. The morphologic investigation of biopsy material confirmed the prior diagnosis. In some months the child died.
This clinical case shows that even in presence of all signs of acute inflammatory process, the dentist must manifest onco-caution. In this case the inflammation, chronic trauma during endodontic manipulations, and also carrying out the physiotherapeutic procedures, in the sprout zone of bone – turned out to be a direct reason, as well as a favorable background for malignant tumor development. At its certain stage an acute inflammatory process appeared, which has masked the oncologic disease.
One more clinical example will show, that rather often the doctors don’t apply the necessary efforts to reveal the reason of the signs of pathology, they don’t use all possible diagnostic methods, don’t consult the colleagues.
The parents noticed in a 5 year old son the mobility of 51 and 81 teeth, what they connected with a natural change of teeth in this age. They saw a doctor, who extracted those teeth. In 4 weeks the 11th and 41st teeth began to erupt. They erupted in conversion with crown parts and the roots diverged. When the teeth partially erupted in this irregular position, the dentist saw them once more. A dental roentgenologic photo (screening) of alveolar sprout was made in area of notified teeth, with the aim to reveal the reason of irregular placement of teeth. As a rule, the reason of it is a middle cyst of upper jaw, or the upper complex – retinated tooth (one or several). On the X-Ray picture neither a cyst, nor a retinated tooth were revealed. The child was begun treatment with the help of removable orthodontic apparatus with the aim to correct the teeth convergence. The treatment lasted from June to September, but not successfully. The central upper constant incisors have erupted, converging with crowns.
When in September the parents addressed to another dentists they revealed the protrusion of upper lip, paste-like liquid on it, a large thickening of alveolar sprout in area of upper frontal teeth, convergence and mobility (II degree) of central incisors (the side incisors were absent and temporary – fell out). On the picture of face skeleton the changes, characteristic for osteoblastoclastoma were observed. The tumor process grasped the alveolar sprout, palate and the bridge of nose. After operation the child was put a denture.
This example helps to arrive at a conclusion that the doctor’s mistake was in unknowing the real reason of teeth eruption. He did not ask for a help of colleagues, did not make X-Ray photo. It must be noted that on dental X-Ray photos it’s very difficult to reveal the pathologic changes in bone, that are characteristic for tumor process, and the observational photos allow doing it rather easily.
Even in case of benign course of tumor process, taking into account the rapidity of tumor growth in child’s age, the replacement tightening or even ruining of neighboring organs by tumors and disturbance of their functions, -the doctor must timely and correctly solve the complex of questions, concerning organization of diagnostic and medicinal aid to a child. In case of rapid growth of a tumor in a child, the observation of its growth, delay of operation – lead to considerable growth of tumors and formation of large defects in jaw-facial area that is not favorable in child’s age.
So, having put the diagnosis to a tumor at a visit to a dentist, or having a suspicion of a tumor, a doctor must immediately direct a child for the next stage of diagnostics and treatment, orienting upon structure of dental service in this region. The principal scheme of connection of dental and oncological service for ensuring prophylaxis, diagnostics treatment and rehabilitation in case of tumors of maxillo-facial area in children looks in the following way:
• a district dentist;
• polyclinics or a dental department, attached to a polyclinics ;
• dental in-patient department or department of tumors of neck and head at oncologic dispensary.
During the choice of treatment tactics, the doctor must take into consideration the hystologic type and a clinical course of the tumor, its localisation, age and a general condition of a child’s health. The choice both of treatment method and a place of its performance in the polyclinics, or
at the dental or an oncologic in-patient department depends on the age, clinical course of the disease (type of a tumor, its localisation and spread) and on the other individual peculiarities of a child.
In early childhood it is more advisable to carry out all kinds of surgical intervention or treatment of neoplasm , applying other methods – at the in-patient department. In elder children – all limited benign tumors may be cured at the dispensary, taking into attention a general condition of children health. All malignant tumors must be treated at the in-patient department. The treatment is carried out according to previously composed plan, which follows the sequence of medicinal measures, their durability, a number of seances, dosage and so on.
In surgical treatment oeoplasm in children a doctor must follow such 2 conditions:
1. Radicality of an operation, because a not complete extraction of malignant and benign tumors leads to speeding of their growth or to recurrency, in which the possibilities of further radical treatment are worsened;
2. Absolute necessity of hystologic investigation of all extracted tumors, even if according to their clinics, macro- and morphologically they don’t case any doubts of their benignity.
It’s necessary to follow the principles of antiblastics and ablastics.
Ablastics-is the prevention of dessimination of tumor cells by way of scaree intrussion on the cell itself ( to avoid traumatezing of the cell, cuttings, pricks) and on surrounding tissues-to prevent the injury of lymphatic ducts, a doctor must protect the operational field, passing from tumorous tissues on surrounding healthy ones, to wash hands; to change gloves and instruments, operational gawn.
Antiblastics-a complex of measures, directed to ruin tumorous cells, which may penetrate into the operational wound, treating it by alcohol (spirit), iodine, applying an electric knife; diathermocoagulation or criosurgical instruments.
The indications for therapy by X-rays or to distant γ –therapy in children are:
• technical impossibility of radical surgical intervention;
• presence of tumors, disposed to postoperational recurrences;
• possibility to cure the tumors without surgical intervention ( with a necessary confirmation of the diagnosis by biopsy methods).
The following preparations are applied for chemiotherapy: antimetabolites, alkilous preparations, antitumor antibiotics, hormonal means and phytopreparations (e.g. fitohematoglutins, which assists to transformation of neutral lymphocytes into killer-cells). Gen-enginering and antisenstherapy are used, that is the influence on a concrete gen by way of syntesis inhibition of the appropriate protein.
At present doctors perform a row of reconstructive operations on lower jaw( mandible). As a rule, the resection of mandible in case of tumor leads to formation of partial or complete defect and that is why it is accompanied by primary or remote bone plastics. In the latter case a postoperational orthodontic treatment is carried out. It’s rather important, that the head of a joint is preserved during resection and to preserve the function of a joint.
In all cases of surgical intervention on upper jaw with further defects the children 2-3 years old must be made dentures 2-3 weeks after operation. The child is discharged from the hospital to be watched by the local orthodontist.
In treatment of malignant tumors the main role belongs to surgical interventions. If to take a group of Yuing sarcomas, and reticular cyst the chemio– and ray therapy may be appropriate.
After a course of treatment at the oncologic in-patient department the children with malignant neoplasms must be constantly examined by dispensary oncologists and dentists. This may help to prevent the progress of a disease, prolong the remission of the disease and a patient’s life. The task of postoperational dispensary observation is the control of possibility of recurrence,renewal of anatomical form, and a disturbed function , ensuring of correct growth and development of jaw-facial bones. That’s why the dispensary care of the patient includes examinations of such specialists as: an orthodontist, pediatrician., logopedist and otolaryngologist.
The clinical, roengenological and hematological control in process of dispensary observation allows to timely reveal the recurrences or the progress of tumor process and to carry out the appropriate preventive therapy, to direct a child to onco-clinics. The repeated courses of therapy by cytostatics, scarcing diet, the all-round strengthening therapy and other recommendations of oncologists are carried out and rendered by place of living of a patient. The dispensarised child at the onset of any respiratory infection, acuteness of periostitis, lymphadenitis, or a tonsilitis must be examined and cured with a special care, as these illnesses may be a result of a main oncopathology.
When the children become 18 years old, they pass to the analogical registration of grown-up persons.
Taking into account the low percent of early diagnostics and the timely visit to a doctor,
low efficiency in treatment of tumors, especially of malignant ones, it is necessary to strengthen a prophylactic work in such directions :
1. Sanitary educational work among population.
2. Formation and support of oncologic warning in all dentists.
3. Increase of level of appropriate qualification of dentists and formation of their knowledge of early symptoms for a timely diagstics of tumors.
4. Improvement of methods of diagnostics and organisation of rendering help to patients in case of slightest suspicion of tumor.
5. Maximally complete involvement by dispanserisation of children with pre-tumorous and chronic processes in jaw-facial area.
So, the oncologic diseases in children have their characteristic features.Only the sufficient knowledge of them as well as the knowledge of oncology theoretical bases in combination with
a clinical mind of a dentist will allow the child dentist to ensure the rendering the proper
specialised help as in organisation of aid, just so – concerning the optimal treatment.
Papilloma refers to a benign epithelial tumor growing exophytically (outwardly projecting) in finger-like fronds. In this context Papilla refers to the projection created by the tumor, not a tumor on an already existing papilla (such as the nipple.)
Nearly 70% are white , but are pink if not keratinized. The affected ages range from 2 -91 years.They are located most frequently on the palate, but also on the tongue, lips of gingivaeWhen used without context, it frequently refers to infections caused by Human papillomavirus (HPV). However, there are other conditions that cause papilloma, such as Choroid plexus papilloma (CPP).
Two types of papilloma often associated with HPV are “squamous cell papilloma” and “transitional cell papilloma” (also known as “bladder papilloma“). Simple papillomas are not infective and have no tendency to spread by autoinoculation.
Microscopy.
Papillomas have a branching structure consisting of a vascular connective tissue core , supporting a thick hyperplastic epithelium, which may be ortho– or parakeratinized.
Management.
The diagnosis should be confirmed by microscopy. Excision should be curative and recurrence is rare.
Epulis is a tumor-like neoplasm of the connective tissue, which arises from marginal periodontal tissue. It can be caused by the chronic trauma by crown or filling or abnormal localization of the tooth in the dental row.
These are of four types:
1. Granulomatous epulis: A mass of granulomatous tissue forms around a carious tooth, or at the site of irritation by a denture requiring extraction of tooth and scraping away of granulation, a part of which should always be examined histologically to rule out malignancy.
2. Fibrous (fibroid) epulis: It is the commonest type of epulis. It is a simple whorled fibroma arising from the periodontal membrane, present under the gum. Rarely a soft bluish-red, bleeding and rapidly growing fibrosarcomatous epulis occurs. Both require removal of adjacent tooth or teeth and resection of a wedge of bone, including a portion of the gum containing the growth.
3. Giant cell (myeloid) epulis: This is an osteoclastoma arising peripherally in the jaw and presenting under the gum. It is soft, sessile and plum coloured because of vascularity. Ulceration and serious haemorrhage can occur. X-ray shows bone destruction with ridging of the walls (pseudo-trabaculation). Treatment is curettage for small lesions but segmental mandibulectomy with mandibular reconstruction for larger tumours.
*Carcinomatous epulis -and ulcerating, invading the bone and spreading to cervical lymph nodes. A biopsy must be performed to confirm the diagnosis. Treatment remains a commando procedure with reconstruction of the jaw and soft tissue defect using a vascularized composite tissue – skin, soft tissue and bone.
Granulomatous, Fibrous and Giant cell epulis are benign epulis, whereas Carcinomatous epulis is a malignant epulis.
Fibromas are growths that form in a person’s mouth in response to repeated injury, irritation or disease. These elevated areas of mostly scar tissue can appear anywhere inside the mouth, including on the tongue, lips, gums and inner cheeks. They can take months or even years to form.
Most fibromas occur as a result of repeatedly biting a certain area of the mouth. Excessive grinding of teeth (bruxism) and a rare genetic disorder called Cowen’s syndrome also are sometimes responsible for fibromas. Fibromas can occur in people of any age, but are most likely to affect adults. Fibromas affect males and females equally.
Fibromas that are not surgically removed continue to grow. If left untreated, fibromas may grow large enough to displace the teeth or cause other oral health problems. For this reason, surgical removal is recommended.
Fibromas typically appear as small areas of elevated tissue that are pink or white. When these scars are traumatized, they may appear reddish or blue. They tend to have a smooth surface and may be round, oval or elliptical in shape. They may have a stalk-like base or be fixed to the surface.
The size of a fibroma can vary from a few millimeters to about 2 centimeters (1 inch). In some cases they feel firm and hard, but in other cases, they are soft, spongy and easily palpable. They are not typically painful but they may become annoying if not removed.
Treatment
-cutting the lesion from the mouth using a local anesthetic to prevent any pain. The area is then usually closed with stitches. Once removed, fibromas do not normally return, unless the area continues to be irritated.
– in some cases a mouth guard can be prescribed to be worn at night by patients who grind their teeth.
Nasopharyngeal (juvenile) angiofibroma– is a rare benign tumor which consists of immature well-vascularised connective tissue. Nevertheless it has tendency to quick invasive growth with germination into adjacent anatomical areas (anterior cranial fossa, maxilla sinus, eye-socket, fossa pterygopalatinae and fossa subtemporalis ) is characterized by high recurrence frequency.
Fibromatoses are proliferative lesions of connective tissue which infiltrate surrounding tissues, have a strong tendency to recur, but are non- neoplastic and no metastasize. They do not appear to be reactive , but their etiology is unclear. Fibromatoses typically form relatively slow-growing , usually painless, poorly circumscribed tumor-like masses.They may be entirely within soft tissues such as muscle or beneath periosteum and cause bone erosion.Later, involvement of nerve fibers can cause pain.
Treatment should be wide excision , but mutilating operations should be avoided. The recurrence rate is less than 25%.
Desmoplastic fibroma is another form of fibromatosis. It is arised from the tendon and fascia- sheath structures. It is aggressive and progressive but does not metastasize.
Lipoma is a benign tumor composed of fatty tissue. These are the most common form of soft tissue tumor. Lipomas are soft to the touch, usually movable, and are generally painless. Many lipomas are small (under one centimeter diameter) but can enlarge to sizes greater than six centimeters. Lipomas are commonly found in adults from 40 to 60 years of age, but can also be found in children.
There are several subtypes of lipoma:
Angiolipoma
Angiolipoleiomyoma
Neural fibrolipoma
Chondroid lipoma
Spindle-cell lipoma
Pleomorphic lipoma
Intradermal spindle cell lipoma
Hibernoma
The most common type is the “superficial subcutaneous lipoma“, i.e. just below the surface of the skin.Most occur on the trunk, thighs and the forearms, although they may be found anywhere in the body where fat is located.
Lipoma of the corpus callosum is a rare congenital condition which may or may not present with symptoms. Lipomas are usually relatively small with diameters of about 1-3 centimeters,but in rare cases they can grow over several years into “giant lipomas” that are 10-20 cm across and weigh up to 4-5 kilograms. Approximately one percent of the general population has a lipoma.These tumors can occur at any age, but are most common in middle age, often appearing in people from 40 to 60 years old. Cutaneous lipomas are rare in children, but these tumors can occur as part of the inherited disease Bannayan-Zonana syndrome.
Usually, treatment of a lipoma is not necessary, unless the tumor becomes painful or restricts movement. They are usually removed for cosmetic reasons, if they grow very large, or for histopathology to check that they are not a more dangerous type of tumor such as a liposarcoma.Lipomas are normally removed by simple excision. This cures the majority of cases, with about 1-2% of lipomas recurring after excision.
A rhabdomyoma is a benign tumor of striated muscle. Cardiac rhabdomyomas are the most common primary tumor of the heart in infants and children.
It has an association with tuberous sclerosis.
It is most commonly associated with the tongue and heart, but can also occur in other locations. Rhabdomyomas are well circumscribed or encapsulated and consist of large cells with eosinophilic cytoplasm.
The malignant form of rhabdomyoma is called rhabdomyosarcoma.
A leiomyoma (plural is ‘leiomyomata‘) is a benign smooth muscle neoplasm that is not premalignant. They can occur in any organ, but the most common forms occur in the uterus, small bowel and the esophagus, in the derm of the tongue back. The tumor growth slowly , but sometimes it can achieve a quite large size. Clinically it is represented as circumscribed node or nodes. It has solid elastic consistency, smooth surface, clear borders. It could be painful during palpation. The skin and mucosa are without changes.
The surgical excision is the choice of the treatment.
Vascular tumors of Jaw-Facial area.
Hemangioma is the most common type of vascular anomaly (birthmark). It is a benign (noncancerous) tumor of the cells, called endothelial cells, that normally line the blood vessels. In hemangiomas, the endothelial cells multiply at an abnormally rapid rate.
Hemangioma may be:
– In the top skin layers (capillary hemangioma)
– Deeper in the skin (cavernous hemangioma)
– Branchy hemangioma
– Mixed hemangiomas
Infantile hemangiomas have a fairly predictable pattern of growth. Most appear during the first weeks of life and grow rapidly (called the proliferative phase) for 6 to 12 months. Then they begin a much slower process of shrinking, or regressing (called the involuting phase), which may take from one to about seven years. Finally, the tumor enters its final, shrunken state (called the involuted phase), after which it will never regrow. Tumor regression is complete in 50% of children by age 5 and in 70% of children by age 7. By the time a child reaches 10 to 12 years of age, involution of the tumor is always complete. Some residual fatty tissue or thin skin may remain after involution.
A tumor near the skin’s surface is called a superficial hemangioma. It often looks like a raised bright red patch, sometimes with a textured surface (hence the once-commonly used term “strawberry hemangioma“). Veins radiating from the tumor may also be visible beneath the skin. As the hemangioma begins to or shrink, the red color fades. Usually, the last traces of color are gone by the time the child reaches age 7.
Hemangiomas that grow in the lower layers of the skin, called deep hemangiomas; they appear bruise-like or bluish or may not be visible at all. They are usually found at two to four months of age.
Congenital hemangiomas look different than the more common type that grows after birth. They are large at birth, round or oval and have a grayish cast with prominent veins and may be encircled by a pale halo.
The most often hemangioma’s localization are chicks, nasal lateral surface, naso-labial sulcus, lips, infraorbital area, floor of the oral cavity.
Capillary hemangioma– is an excrescence of dilatating and twisting capillaries and small vessels which are deeply intertwined and have a lot of anastomosis. During the palpation capillary hemangiomas are painful. They are getting paleness while pressing and renew the color after ending the pressure. There are two forms of capillary hemangiomas : the flat ( doesn’t arise under the skin) and the hypertrophic one ( arise under the skin and has hilly surface ) .
Branchy hemangioma consists of twisted thick-walled vessels of arterial or venous type. It is rarely occur in the oral-facial areas and considered as developmental malformation of large vessels. Clinically it is represented as a single node with tense pulsation that becomes weak after the vessel‘s squeezing. During an inclination of a head, the tumour is increased at a rate of size and after returning in target position the tumor decreases( so-called filling- devastation symptom).
Cavernous hemangioma is a group of cavities filled with blood that have endothelium lining which are incorporated or divided by connective tissue partitions. During diagnostic puncture the venous blood is obtained and long-lasting haemorrhage can occur.
Mixed hemanhgiomas are the association of all types of hemangiomas written above.
There are no ways to prevent hemangioma. Nothing the mother does or does not do prior to or during pregnancy plays any role in whether her child develops a hemangioma.
While complications are uncommon, they can occur in some children. Complications include ulceration (skin breakdown), which can bleed or become infected; obstruction of vital functions such as vision, hearing, or breathing; distortion of facial features; and, very rarely, internal bleeding or high output cardiac (heart) failure resulting from a hemangioma in an internal organ. Only about 1% of hemangiomas cause life-threatening complications.
About 5 percent to 10 percent of children with a hemangioma develop an ulcer, typically on the lip or the peri anal or genital region. An ulcer is usually effectively treated with topical antibiotics and frequent cleansing and dressings. Sometimes oral medications, laser treatment, or surgical removal may be necessary. Bleeding, which is rare, can usually be controlled by applying pressure to the area. An ulcer usually heals within a few weeks and does not recur. However, it may result in scarring that requires surgical correction.
Treatment should be given when the benefits of treatment outweigh the risks involved. All treatments have side effects.
Most haemangiomas disappear by themselves and do not need any treatment.
Sometimes a small biopsy of skin is taken in order to make sure what type of haemangioma it is.
Haemangiomas need treatment if:
- They are pressing on important structures such as the eyes, nose, ears or windpipe.
- It is on your child’s eyelid or if it is large and on your child’s face. These haemangiomas need urgent treatment to prevent serious problems.
- They become ulcerated and painful and do not heal.
- To prevent your child having problems with their appearance when they reach school age.
Methods of treatment :
– medical dispensary observation;
– surgical treatment;
– tumor embolization;
– sclerotherapy;
– laser;
– criotherapy and electrocoagulation;
– steroids and interferon medicines
Surgery therapy includes:
– dermabrasion;
– excision ;
– suture ligation of the vessels;
– subcutaneous devastation ;
– combination of all listed methods above.
Lymphangioma is a rare, benign, congenital disorder in which a blockage in the lymphatic system causes fluid to accumulate in bubbles beneath the skin.
These proliferation of lymph vessels may occur anywhere, but around 75% occur in the head and neck regions, or in the groin. Around 90% are either evident at birth or become evident before age two years. Since they have no chance of becoming malignant, lymphangiomas are usually treated for cosmetic reasons only.
Vascular malformations include a wide variety of lesions which are very different from hemangiomas. They are true developmental abnormalities of the vessels involved so they are always present at birth, even though they may not be apparent until later. They always grow over the lifetime of the individual and never regress. Some grow very slowly, others rapidly but they all get worse over time to some degree. The malformations are classified by the vessels involved. So, there are venous malformations, lymphatic malformations, arterial malformations or combinations (such as arterio-venous malformations).
Port Wine Stains (PWS) are a common type of malformation. They are technically a venular (small veins) malformation but the term port wine stain is so ingrained in our language, and the birthmark is not easily confused, that it is acceptable to keep this term. They are quite common, occurring in about 0.3% of the population with an equal distribution among males and females. Like all malformations, they are present at birth and get worse, to some degree, over time. They can be pale pink to dark purple in color and their cause is thought to be related to a problem in the nerve regulation of the vessels – though this is not proven.
Capillary Malformations are flat, pink-red birthmarks that are most common on the face and neck. Most commonly these are the lesions associated with Sturge-Weber syndrome. These malformations should not be confused with the very common staining seen iewborns on the forehead, nape of neck and eyelids. These very predictably fade in the first year while capillary malformations do not.
Venous malformations can be deep, superficial, diffuse or localized. They commonly appear as bluish, spongy masses that can be compressed easily. When the child is lying down or crying, the lesion fills up with blood and the mass becomes fuller. Occasionally, hard lumps can be felt in the malformation, these are calcified nodules. The most common areas affected are the cheek, floor of mouth and extremities. Pain and swelling are common complaints. Klippel-Trenaunay Syndrome (KTS) is a venous-lymphatic malformation of the extremities, most commonly of the legs. Venous malformations can get worse during puberty and pregnancy or as a result of infection and trauma. Lymphatic malformations are most commonly found in the neck, cheek or mouth or in association with venous malformations in KTS. In the neck, they are often called ‘cystic hygroma’ but this term should be discontinued. These lesions are made up of expanded lymphatic channels which can be quite large and typically get bigger due to infection, such as a cold, and then go back to their regular size once the infection goes away. There are two major types of lymphatic malformations – macrocystic and microcystic. Macrocystic lesions have large (macro) pockets and microcystic have very small lesions that invade the tissues. It is important to tell these apart for treatment.
Arterio-venous malformations are typically found in the brain; the second most common site is the head/neck most commonly in the mid-face. They are often pulsatile and firm and, like other malformations, always present at birth.
Congenital Cysts and Fistulas of the neck. Etiology. Clinic. Diagnosis. Treatment.
The cervical lymphoepithelial or branchial cleft cyst is a developmental cyst that is usually present as a unilateral, soft-tissue fluctuant swelling that typically appears in the lateral aspect of the neck, anterior to the sternocleidomastoid muscle, and becomes clinically evident late in childhood or in early adulthood.
Types
– First branchial cleft cysts typically originate in the angle of the mandible and extend to the external auditory canal. They are often associated with the the facial nerve
– Second branchial cleft cysts are most common. They are found along the anterior border of the sternocledomastoid, passes through the carotid bifurcation and into the tonsillar pillar
– Third branchial cleft cysts are rare and found in the lateral neck
The branchial apparatus that begins to form in the second week of fetal life and is completed by the sixth or seventh weeks is probably the structure most widely believed to be the source of branchial cysts.
These lesions are found behind the angle of the mandible in the anterior triangle of the neck at the junction of the upper third and lower two-thirds of the sternocleidomastoid muscle. The cysts have a lining of stratified squamous epithelium resting on a complete or incomplete band of lymphoid tissue with part of the cyst wall resembling a lymph node.
Regardless of etiology, it is essential to understand the differences between a cyst, fistula and sinus. This is especially troublesome when speaking of branchial cleft cysts because some have suggested that cysts are wholly different from fistulas and sinuses, in regard to their time of development, clinical presentation, histology and familial tendencies.Some investigators doubt a common etiology between the cysts and sinuses. Many surgeons believe that congenital lateral cervical sinuses and fistulas result from the branchial apparatus; however, it is possible that many mechanisms may lead to branchial anomalies.
It was found in the survey that sinuses and fistulas typically arise in the first decade of life and to a lesser extent in the second decade, whereas cysts occur in the adolescent and adult. Congenitally presented sinus tracts usually are the result of spontaneous or surgical drainage from a cyst and frequently have a familial incidence.5 Microscopically, cysts are lined by stratified squamous epithelium lying on top of lymphoid tissue, with part of the wall resembling a lymph node.
The branchial cleft cyst typically is a slow-growing, fluctuant mass that becomes apparent in the second and third decades of life. A close association with the external ear, angle of the mandible and upper lateral aspect of the neck usually is observed, which is consistent with the most commonly accepted etiology (that is, coincident with the fetal location of the branchial apparatus). The swelling typically develops in the junction between the upper one-third and lower two-thirds of the anterior sternocleidomastoid muscle, but it can occur at any level from the hyoid to the suprasternal notch. The cyst generally is movable in all planes and is motionless during swallowing.If large enough, the anomalies can cause asymmetry of the neck, as well as dyspnea, dysphagia and dysphonia. The swelling usually is unilateral, but cases of bilateral cysts/sinuses have been reported, with an incidence of 2 to 3 percent. When bilateral cysts/sinuses develop, there seems to be a familial tendency.
Inflamed cysts may become abscessed, which eventually can lead to rupture. A permanent sinus may form or recurrent cyst formation and infection may occur.
Diagnosis of branchial cleft cyst. The diagnosis of branchial cleft cyst is made primarily by medical history, clinical manifestations and exclusion. Preoperative ancillary diagnostic procedures include computed tomography (CT), sonography and fine-needle aspiration ( FNA). CT may be particularly useful not only to visualize the full extent of the lesion, but also to delineate its association with adjacent structures.
These lesions are deemed amenable to sonography because of their typically superficial nature. The sonomorphologic findings typically yield a rounded mass that has a uniform low echogenicity lacking internal septation, with no acoustic enhancement or motion. This echogenicity probably is due to the accumulation of cellular material as well as cholesterol within the cyst lumen. Other sonographic reports have noted echogenicity in only the gravity-dependent portions of the cysts.
The use of FNA has been mentioned in the literature as being effective iarrowing the diagnosis when a lateral neck lesion is present. Aspirate appears as a straw-colored fluid that microscopically may exhibit squamous cells, polymorphonuclear cells, lymphocytes and cholesterol crystals.
TREATMENT
Surgical excision of the branchial cleft cyst is the treatment of choice and is considered definitive. Some controversy exists about when to operate and whether every cyst requires removal. Many surgeons believe that presence of the lesion is reason enough for removal, primarily because of its propensity for infection. Excision of an asymptomatic lesion precludes or minimizes the chance of infection complicating a later surgery.
Any existing infection must be controlled before surgery can be performed, and can be achieved with antibiotics, with or without incision and drainage. Although incision and drainage may be necessary to treat an infective episode before surgery, it is not recommended as definitive treatment. Furthermore, some authors have suggested that complete drainage of lumenal contents before surgery is not desirable because this may make dissection more difficult. Infection doubles the recurrence rate of these anomalies, as do incomplete dissections and non-curative procedures. Approximately 80 percent of branchial sinuses will open to the skin, and fewer will open to the pharynx. These sinuses initially may suggest a cyst, but on surgical exploration prove to be sinuses with a tract leading medially. Clinicians must take care to remove the entire tract to decrease the chance of recurrence. Surgical complications include injury to surrounding structures such as the carotid sheath and the spinal accessory and hypoglossal nerves. Other treatment modalities that have been reported are radiation therapy, repeated incision and drainage, and use of sclerosing agents. These are considered noncurative, and if performed before surgery can increase the recurrence rate after surgical excision.
The midline(thyroglossal) cysts and ducts of the neck belong to a developmental malformations of the branchial apparatus.
Embryology — The analage of the thyroid gland forms at the foramen cecum of the tongue, which is located on the dorsum of the tongue posteriorly at the apex of the V-shaped sulcus formed by the circumvillate papillae. During the fourth week of gestation, a ventral diverticulum of the foramen cecum is formed from the first and second pharyngeal pouches (the medial thyroid analage). This diverticulum, with its narrow neck connected to the tongue, descends in the midline of the neck as the thyroglossal tract to the position of the normal thyroid in the base of the neck, where the thyroid lobes separate, by the seventh week. The path of descent is usually anterior to the hyoid bone, but may be posterior to or through the bone, and ends on the anterior surface of the first few tracheal rings.
The congenital cysts and ducts of the neck are observed in 1 person on 3000 newborns. Clinically they are manifested at 4 till 7 years old or at 10-14 yeas that can be connected with hormonal changes in the child’s organism. The primary ducts are connected with oss hyoideus and foramen cecum linguae ( cecal foramen of tongue). Secondary ones are formed due to suppuration of midline cysts.
The child or parents complain of existence of painless node on midline of the neck which long exists , is gradually increased in sizes, can suppurate . When midline ducts are present there are complaints on existence of “points” with mucus-like content which is going through it.
Clinically.
The most common locations for a thyroglossal cyst is midline or slightly off midline, between the isthmus of the thyroid and the hyoid bone or just above the hyoid bone. A thyroglossal cyst can develop anywhere along a thyroglossal duct, though cysts within the tongue or in the floor of the mouth are rare.
The midline cyst is determined in the anterior neck projection as a tumor-like round-shaped neoplasm with the precise borders, as dough-like or smooth consistence which is displaced during swallowing together with the body of hyoid bone. It happens due to connection between the cyst and the body of the hyoid bone. If the cyst is connected with oral cavity, its size can periodically decrease after releasing of the cysts content into the oral cavity. When the cyst suppurates the pain and the hyperemia of the skin can occur. This inflamed cyst reminds abscess.
Treatment.
The surgical excision is the choice of treatment. It is necessary to mention that during of cystectomy the resection of the part of the body of oss hyoideus should be provided. The drainage should be done in case of cyst suppuration.
The lateral cysts and ducts of the neck.
A preauricular sinus or cyst (also known as a “Congenital auricular fistula,” “Congenital preauricular fistula,” and “Ear pit”) is a common congenital malformation characterized by a nodule, dent or dimple located anywhere adjacent to the external ear.They are inherited features and usually appear on one side, but may be bilateral in 25-50% of cases. Preauricular sinuses and cysts result from developmental defects of the first and second branchial arches. Occasionally a preauricular sinus or a cyst can become infected.
Most preauricular sinuses are asymptomatic and remain untreated unless they become infected too often.Preauricular cysts are treated with surgery which, because of their close proximity to the facial nerve should be left to an experienced surgeon.
