Tumors

Tumors of the salivary glands. Plastic removal of salivary fistulas. Benign tumors of soft tissues. Vascular tumors and related patches. Immunological concept of tumor development

The salivary glands consist of three major paired glands (the parotid, submandibular, and sublingual) as well as numerous minor salivary glands, situated mostly in the oral cavity but also found in the pharynx, larynx, trachea, and sinuses. In the oral cavity 700 to 900 minor salivary glands are found, the majority of which are located at the junction of the hard and soft palates. These glands produce saliva, which functions as a lubricant for speech and swallowing, assists taste, has antibacterial and immunologic properties, and contains digestive enzymes. The salivary glands are affected by many different disease processes, some of which are surgical in nature while others have a medical basis. Surgical diseases include tumors, stones, and cysts, whereas medical diseases include viral infections, autoimmune diseases, and sarcoidosis. This chapter will concentrate on the salivary gland diseases that are of most interest to the surgeon.

Tumors Tumors of the salivary glands show a wide variety of pathologic types varying from benign to highly malignant. Salivary cancers are comparatively rare and comprise 3% of head and neck cancers, which in turn account for 3% of all malignancies. These neoplasms will be discussed according to their histopathologic diagnosis and the surgical principles dictated by the site of the tumor.

 Histopathology The large variety of tumors that occur in the salivary glands make an exhaustive list of all types impossible in a chapter of this length. The most common epithelial salivary gland tumors will be reviewed in order to illustrate the fundamentals of management of salivary neoplasia.

 Benign Tumors

Pleomorphic Adenoma The pleomorphic adenoma is the most common benign salivary tumor at all sites. Approximately 80% of all pleomorphic adenomas (PSAs) occur in the parotid, and despite their slow growth they can become extremely large if neglected. This tumor is thought to arise from both salivary ducts and myoepithelial cells and is a true “mixed tumor.” Because of its derivation, histologically, many different patterns can occur, from cellular, glandular, and myxoid types to cartilagenous and even ossified forms. These features can be seen in different areas of the same tumor, accounting for its name, pleomorphic (Greek for many forms). The important feature from a surgical standpoint is the presence of a “pseudo capsule,”which contains outgrowths or pseudopodia of the tumor. Attempts at “enucleation” of the tumor from within its “capsule” will inevitably leave viable tumor cell nests and predispose the patient to multifocal recurrence. Some authorities believe that younger patients with pleomorphic adenomas have a higher chance of tumor recurrence and increased growth during pregnancy. Malignant change is rare and usually takes place in long-standing tumors, the most common type being carcinoma ex pleomorphic adenoma. Prognosis will depend on the type of malignancy and involvement of the capsule. Rarely, malignant change in both elements of the pleomorphic adenoma (ductal and myoepithelial) will occur giving rise to the carcinosarcoma or true mixed malignant (biphasic) pleomorphic adenoma. On rare occasions, an apparently histologically benign tumor will metastasize into the so-called benign metastasizing pleomorphic adenoma.

 Warthin’s Tumors This benign tumor is almost exclusively found in the parotid. It occurs mostly in men and is more common in smokers. It is thought to derive from salivary duct cells that are entrapped in lymph nodes during embryonic development. The tumor consists of large cystic spaces with a surrounding columnar epithelium and a stroma of lymphocytes. Surgically these tumors may be multiple in one parotid gland or bilateral, or involve lymph nodes adjacent to the parotid gland.1,2

 Hemangioendothelioma In children the most common cause of parotid mass is a hemangioma or hemangioendothelioma.3 These are benign tumors that may appear soon after birth and grow rapidly. Usually, conservative treatment while waiting for involution is recommended.

 Malignant Tumors

Mucoepidermoid Carcinoma Mucoepidermoid carcinoma (MEC) is the most common malignant salivary gland neoplasm in both adults and children, and the most common salivary gland cancer of the parotid and minor salivary glands. This tumor can be of low grade or high grade depending on its histology. Low-grade MECs have multiple macrocysts and abundant mucus-producing cells. High-grade varieties have multiple squamous cells and very few mucus-producing cells or cysts, and mucicarmine or periodic acid–Schiff stains may be needed to identify intracellular mucus to characterize this tumor. There are three cell types of MEC: mucus producing, intermediate, and squamous. The respective ratio of mucusproducing cells to squamous cells will determine the clinical aggressiveness of the tumor (see above). Low-grade MECs can be very slow growing and nonmetastasizing, and can generally behave like a benign tumor. High-grade MECs can exhibit aggressive growth and invasion resulting in widespread metastasis and death. Highgrade tumors usually show increased pleomorphism and meiotic figures. High-grade lesions may metastasize to cervical lymph nodes or spread hematogenously to the lung, liver, and bone. Adenoid Cystic Carcinoma Although this tumor is very slow growing, its relentless course,with repeated recurrence and metastasis via the blood stream, gives low 20-year survival rates.4 Adenoid cystic carcinoma is the most common malignancy of the submandibular gland and is the second most common salivary gland cancer overall. Three histologic types are seen: tubular, cribriform (the classic “Swiss cheese” pattern), and solid. The solid type has the worst prognosis, especially when areas of necrosis are present. The infiltrative nature of this lesion and the frequency of perineural involvement with spread along the nerve mandate wide resection margins. Perineural spread is a bad prognostic sign for both local recurrence and distant metastasis. Clinical and radiologic examination of this tumor frequently underestimate its true extent, and follow-up of 15 to 20 years is required as late recurrences occur.

 Low-Grade Polymorphous Adenocarcinoma Low-grade polymorphous adenocarcinoma occurs almost exclusively in the minor salivary glands and is second only to mucoepidermoid carcinoma at these sites. It arises from terminal duct cells and is characterized by cytologically bland monotonous cells that can assume many different patterns (glandular, cribriform, and lobular) within the same tumor. Characteristically “Indian file” cells and perineural involvement are seen. Although this tumor behaves in a very lowgrade manner, local recurrence will occur with inadequate excision.5 The important pathologic features seen from the surgeon’s viewpoint are frequent misdiagnosed on initial biopsy, due to the different patterns that may be sampled.Common misdiagnoses are adenoid cystic carcinoma, pleomorphic adenoma, and malignant pleomorphic adenoma. It is also important to be aware that the frequent presence of perineural involvement does not lead to a worse prognosis, as is the case for adenoid cystic carcinoma.

Site of Tumor Parotid Gland The surgical principles of treating parotid tumors are dictated by the histopathology of the tumor and the need to preserve the facial nerve. Diagnostic imaging with computed tomography (CT) or magnetic resonance (MR) is desirable for superficial lobe tumors but is essential for suspected deep-lobe neoplasms, especially those with a parapharyngeal component. Since 80% of parotid tumors are benign and 80% of these are pleomorphic adenomas, a solitary mass in the parotid with no features of malignancy is most likely a PSA. Open biopsy of such a mass is therefore contraindicated as this will rupture the “capsule” and “seed” the PSA, increasing the complexity of subsequent surgery and chances of recurrence. Fine-needle aspiration biopsy (FNAB) for cytology is the preferred method of diagnosis.6 Clinically only one-third of malignant tumors will have symptoms or signs of malignancy, such as pain, ulceration of skin, facial nerve palsy, or metastatic cervical nodes.7 Thus virtually all parotid tumors will initially be treated as benign unless FNAB shows definite malignancy or there is clinical evidence of malignancy (Figure 35-1). The majority of tumors occur in the superficial lobe, and superficial lobectomy with preservation of the facial nerve has been the standard operation for many years. Recent minor modifications have included the use of a face-lift incision, the use of the superficial musculoaponeurotic system to prevent Frey’s syndrome, the use of flaps or alloplasts to augment defects, and the suggestion that “capsular dissection” without the need to remove the entire superficial parotid may be sufficient. 8–10 Superficial lobectomy is suitable for benign and low-grade malignant tumors, and even in high-grade malignancies only branches of the nerve that are actually infiltrated will be sacrificed. If the nerve or portions of it have to be resected, immediate grafting is recommended. In deep-lobe tumors a total parotidectomy is performed, with the superficial lobe being dissected first to expose the nerve. Good margins with surrounding normal salivary gland tissue are more difficult to obtain on deep-lobe tumors, which tend to be large as they are often detected late. In high-grade tumors, surrounding tissues such as skin, masseter, and mandible may require sacrifice, as dictated by the need to obtain clear margins. In these instances consideration should be given to neck dissection.Where clinically positive nodes are present, a modified radical neck dissection is usually the operation of choice.11 Where the patient is N0 clinically, but at high risk for occult nodal disease, a selective neck dissection of levels I to IV or levels II to IV is indicated. In high-grade tumors postoperative radiation therapy is usually indicated. Chemotherapy has not been shown to convey a survival benefit for these lesions.

 Submandibular Gland In suspected submandibular neoplams, CT imaging, MR imaging, and FNAB are all useful in the diagnostic work-up. Fifty percent of tumors will be malignant, adenoid cystic carcinoma being the most common. In benign neoplasms (PSAs) removal of the submandibular gland with an extracapsular dissection of the tumor and 2 to 3 mm of surrounding soft tissue is sufficient. For malignant tumors the minimum resection will be an en bloc removal of level I. If indicated the overlying platysma superficially and the mylohyoid muscle deeply will be excised. In most malignant tumors with N0 necks, the cervical incisioecessary for removal of level I will dictate extending this to a supraomohyoid neck removing levels I to III. The adenoid cystic carcinoma does not usually metastasize via the lymphatics; instead it spreads hematogenously and neck dissection may not be indicated. The mandibular branches of the facial, lingual, and hypoglossal nerves are all in close relation to the submandibular gland. If these nerves appear to be involved by cancer, they should be traced until the nerve appears normal.After resection, frozen sections should be sent from the cut nerve trunk to confirm clearance, although “skip” lesions do occur. Radiation may be useful postoperatively.

 Minor Salivary Glands The Palate The majority of minor salivary gland tumors occur at the junction of the hard and soft palates. In this location 50% are malignant, the most common being low-grade mucoepidermoid carcinoma followed by low-grade polymorphous adenocarcinoma. Coronal and axial CT scans with bony windows are helpful to demonstrate bone destruction and involvement of the sinuses or nasal cavity. Biopsy through the middle of the lesion is indicated as the overlying mucosa will be excised.12 In PSA, excision with a 5 mm margin is adequate. The periosteum is a good deep margin if the bone is uninvolved, as is usually the case with PSA (Figure 35-2). In low-grade lesions a 1 cm margin and similar approach

 

are used. Local flap reconstruction or the use of a palatal plate with subsequent secondary healing by granulation is used for reconstruction. Where bone invasion has occurred, as in adenoid cystic carcinoma or high-grade tumors, a partial maxillectomy will be required. In the case of adenoid cystic carcinoma, attention must be given to the greater palatine nerve, with frozen section clearance obtained. Cranial extension, orbital involvement, and infiltration posteriorly into the pterygoids will increase the extent of surgery and its morbidity, with a decrease in survival (Figure 35-3). Reconstruction is usually with an obturator, although primary maxillary reconstruction has been revisited with the development of interosseous implants and composite microvascular free flaps. The Retromolar Fossa Although this is a relatively unusual site for minor salivary gland tumors, virtually 100% are malignant and are low-grade mucoepidermoid carcinomas. The surgeon should be aware that a cystic soft tissue mass distal to the third molar, with or without radiographic mandibular involvement, is unlikely to be a mucocele, and incisional biopsy should be undertaken to confirm the diagnosis.

 Intrabony Tumors Although intrabony (central) salivary gland tumors are rare, the vast majority are malignant lowgrade mucoepidermoid carcinomas.13 These are mostly seen in the third molar region of the mandible and are frequently multilocular. The tumors are often diagnosed radiologically as ameloblastomas, or odontogenic keratocysts. Resection with a 1 cm margin and sacrifice of the inferior alveolar nerve and overlying soft tissue in areas of perforation are required. Neck dissection is usually not necessary, but if the neck has been opened widely for mandibular resection a supraomohyoid neck dissection can be undertaken. A reconstruction plate is placed and either primary reconstruction with a fibular or deep circumflex iliac artery microvascular flap or secondary posterior iliac crest corticocancellous reconstruction may be used.

Other Intraoral Sites Interestingly, the proportion of benign to malignant tumors varies according to site, with virtually all upper lip tumors being benign and a higher proportion of lower lip tumors being malignant. Salivary gland neoplasms of the tongue and buccal mucosa tend to be malignant and require wide soft tissue dissection to obtain margins.

The Sublingual Gland Less than 1% of all salivary gland tumors occur in the sublingual gland but almost 100% are malignant. Surgical approach will be dictated by the histology and required access for margins. In most cases we have preferred a lip split and mandibulectomy to allow good visualization of the tumor, direct examination of the mandibular lingual cortical plate, and the ability to trace back the lingual nerve when necessary.

 Obstructive Disease Obstruction to the salivary glands is usually seen in the submandibular and parotid glands. It may be due to calcified stones (most common in the submandibular gland) or mucous plugs (most common in the parotid) or strictures of the duct. Stone formation is classically due to stasis of flow, infection, and alteration of the duct contents. Calcified stones are formed by the precipitation of calcium salts around a nidus of mucous plugs, epithelial cells, or microorganisms. Approximately 80% of sialoliths occur in the submandibular gland.14 Microliths in the minor salivary glands have been described. As calcified sialoliths increase in size they may give rise to symptoms, especially when they are present in the duct. Classically the patient reports pain and swelling when eating or drinking or sometimes even from the smell of food (Figure 35-4). Examination of the gland may show a tender swelling with inability to milk saliva from the duct orifice. Plain radiography is used to demonstrate calcified stones, the lower occlusal film for the submandibular gland, and an occlusal or periapical dental film held in the cheek for the parotid. Lateral oblique mandibular films or panoramic radiographs will show parotid duct stones and calcified stones in the hilum or glandular substance of the submandibular gland. CT

 

scans and ultrasonography have also been used. When a noncalcified (mucous plug) obstruction is suspected, sialography may demonstrate a filling defect (Figure 35-5). Acute infection should be managed with antibiotics prior to sialography. Treatment of the stone will depend on its location.

Submandibular Gland Anterior Duct If the stone is palpable in the anterior floor of the mouth close to the orifice of Wharton’s duct, an intraoral approach may be used. Although the anterior duct is traditionally regarded as a line between the first molars, the floor of the mouth slopes downward following the mylohyoid muscle as the premolars are reached, and technical difficulty is increased as the stone is more distal (Figure 35-6). Initially a suture is passed behind the sialolith around Wharton’s duct to use as a traction suture, tenting the duct upward and preventing posterior displacement of the stone during surgical manipulation. An incision in line with the duct is made through the mucosa and dissection carried down to the duct. This is opened in its long axis allowing removal of the stone. The posterior suture is removed and the gland is milked or explored with a lacrimal probe to find other stones. The duct is sutured open to the edges of the mucosa (fish tailed) to prevent stricture.

 Posterior Duct Stones in the posterior submandibular duct are much more technically difficult to remove intraorally, requiring general anesthesia, excellent light, and retraction, as well as the help of an assistant to push the gland upward into the mouth from extraorally. Even so, irritating bleeding can occur and the lingual nerve must be visualized and protected (see Figure 35-6).

 Stones in the Hilum or Gland When the stone is below the posterior edge of the mylohyoid muscle, removal of the gland is necessary. Although intraoral submandibular gland excision has been described, the potential for bleeding from branches of the facial vein and artery and possible scarring of the anterior pole of the gland to the mylohyoid muscle can make this a technically challenging and hazardous procedure.15 We believe that the conventional cervical approach gives the best access and is the safest procedure. Under general anesthesia an approximately 5 cm incision is made over the submandibular gland at 11⁄2- to 2-finger breadths below the mandible. This incision should be parallel to the neck skin creases, not to the lower border of the mandible (Figure 35-7). The platysma is sectioned and the inferior pole of the submandibular gland visualized. The gland is exposed by subcapsular dissection at the inferior posterior pole. Blunt finger dissection will release the deep surface of the gland. The authors do not routinely tie the facial artery and vein at this stage as these can usually be dissected off the gland, although clipping multiple arterial branches to the gland can be tedious. The anterior pole of the submandibular gland is mobilized off the mylohyoid muscle, and in cases of chronic sialadenitis, sharp dissection may be necessary due to dense fibrosis. The superior pole of the gland is dissected in a subcapsular plane and the gland mobilized posteriorly. The posterior edge of the mylohyoid muscle is retracted to expose the lingual nerve and the branch to the gland is tied and sectioned (see Figure 35-7B). The submandibular duct is dissected superiorly into the floor of the mouth as far as possible, tied, sectioned, and the gland removed. Parotid Gland Most obstructive symptoms in the parotid gland are associated with noncalcified stones or mucous plugs. Although these can sometimes be removed with tweezers following duct dilatation or “milked” from the duct, they often cause repeated bouts of pain and swelling. Sialography is helpful in evaluating the extent of damage to the ductal architecture. Sialograms may show changes varying from mild sialectasis to gross dilatation of Stensen’s duct with loss of secondary and tertiary ducts (Figure 35-8). Sialograms are frequently helpful symptomatically, with cure or improvement in many patients. In advanced cases with no improvement, parotidectomy may be required.

 Stones in the Terminal Duct Radiographically opaque stones at Stensen’s papilla can be managed intraorally in a similar manner to those of the anterior portion of Wharton’s duct. Following placement of a posterior traction suture, the duct is opened with an incision running in the long axis of the duct.

 Stones in the Posterior Duct When the stone involves the extraglandular portion of the duct lateral to the buccinator muscle, both intraoral and extraoral approaches are described.16,17 The intraoral approach involves a Y-shaped mucosal incision, dissection through the buccinator muscle, and the use of a traction suture to pull the duct into the mouth. The extraoral approach requires the duct to be displaced laterally with a finger placed in the mouth, with blunt dissection down to the stone, avoiding the facial nerve.

 Parotid Gland Stones Stones at the hilum of the gland or intraglandular stones usually require a parotidectomy if they are symptomatic. The facial nerve dissection may be challenging due to extensive fibrosis (Figure 35-9).

Nonsurgical Approaches Miniature endoscopes have been used to visualize sialoliths and remove them with baskets.18 Lithotripsy has also been attempted either via endoscopes (intracorporeal) or extracorporeally. Intracorporeal lithotripsy uses shock waves produced by lasers, electrohydraulic sources, or a pneumoballistic source. In a review of 6 series of extracorporeal lithotripsy ranging from 33 to 104 stones, Escudier reported a stone-free range of 18.2 to 52.9% with residual fragments occurring in 47.1 to 81.8% of cases.19

 Mucoceles and Ranulas Mucoceles are mostly due to extravasation of mucus from a salivary gland, although a few are true retention phenomena. The most common site is the lower lip, due to trauma (usually following an accidental bite in a child). Mucoceles are simple to treat and they should not recur if the underlying damaged minor salivary gland has been removed. Following a vertical incision through the mucosa over the mucocele, a number of minor salivary glands are usually identified. As it may be impossible

 

to identify the damaged gland, all these minor glands should be removed before carefully suturing the mucosal incision. Ranulas are large retention phenomena that occur in the floor of the mouth in relation to the sublingual gland. They may be large enough to elevate the tongue and interfere with speech and swallowing (Figure 35-10).Where dehiscence in the mylohyoid muscle occurs, the mucus can drain into the submandibular space as a “plunging ranula.” The treatment of ranulas has been reviewed at length in a classic paper by Catone.20 He concluded that definitive therapy was removal of the sublingual gland. Several large series have been reported comparing sublingual gland excision with so-called marsupialization, demonstrating 100% cure for gland excision and 43 to 63% cure for marsupialization.21,22 Despite this evidence some authorities still plead the case for marsupialization or “marsupialization with packing,” which they claim has a lower recurrence rate of 10 to 12%.23 We subscribe to the view that ranulas should be treated by sublingual gland excision. An intraoral approach is made with an incision along the axis of the gland lateral to the ductal orifices. The submandibular duct is identified, either by dissection or following cannulation with a lacrimal probe. The gland is dissected in a subcapsular plane with meticulous hemostasis.At its posterior pole the lingual nerve is identified as it crosses the duct and is preserved. The sublingual gland is dissected from anteriorly, and the final excision is the posterior pole after visualizing the lingual nerve.

Benign tumors of the soft tissues of Jaw-Facial area of Childhood.

 

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.