soft tissue swellings 3.pptx

Soft Tissue Swellings 3 Benign Neoplasms and Reactive Lesions Giant Cell Fibroma Giant Cell Fibroma Clinical Features: Giant cell fibromas usually present as asymptomatic, sessile or pedunculated nodules less than one centimeter in size. While they may have a smooth surface, they oft...

Soft Tissue Swellings 3 Benign Neoplasms and Reactive Lesions Giant Cell Fibroma Giant Cell Fibroma Clinical Features: Giant cell fibromas usually present as asymptomatic, sessile or pedunculated nodules less than one centimeter in size. While they may have a smooth surface, they often have a papillary surface. Sixty percent are diagnosed during the first three decades with a slight female gender predilection. Fifty percent occur on the gingiva and the mandible is more commonly affected site of this lesion (2:1). Giant Cell Fibroma Histological Features: The mass is composed of vascular, loosely arranged fibrous connective tissue. The hallmark of the lesion is the presence of large, stellate fibroblasts, which may contain several nuclei, within the fibrous connective tissue. The covering epithelium is often thin and atropic and the rete ridges may be elongated and narrow. Lesions with a similar histological appearance found on the lingual mandibular gingiva in the region of the mandibular canine, have been called retrocuspid papillae. Giant Cell Fibroma Treatment and Prognosis: Treatment consists of conservative surgical excision. If properly excised, the lesion rarely recurs. Fibromatosis and Myofibromatosis  Fibromatosis and Myofibromatosis Introduction: Fibromatoses are a broad group of fibrous proliferations with a biological behavior ranging from benign to malignant. Myofibromatosis is a similar but less aggressive proliferation myofibroblasts.  Fibromatosis and Myofibromatosis Clinical Features: Fibromatosis presents as a firm, painless mass, which may grow rapidly or slowly. Fibromatosis is more common in children and young adults with a mean age between 8 and 11 years. The site predilection for fibromatosis is the paramandibular soft tissues. These lesions can grow to a large size and can destroy bone.  Fibromatosis and Myofibromatosis: Clinical Features Cont.: Myofibromatosis is seen most commonly in neonates and infants. It commonly arises as a firm mass in the dermis or subcutaneous tissues of the head and neck. Cases of intraosseous myofibromatosis have been reported. While these lesions are usually solitary, multiple lesions in the same patient have been reported. Fibromatosis and Myofibromatosis Histopathology: Unencapsulated, solid, nodular mass of dense, hyalinised connective tissue which haphazardly arranged. Mild chronic inflammatory cells. The surface epithelium may be hyperkeratotic, hyperplastic or atrophic and ulcerated. Fibromatosis Myofibroma  Fibromatosis and Myofibromatosis Treatment and Prognosis: Fibromatosis is treated by wide surgical excision because these lesions are typically locally aggressive. Oral/paraoral fibromatosis has approximately a 23 % recurrence rate. Myofibromatosis can be treated by local excision; cases of spontaneous regression have been reported. Multicentric lesions of myofibromatosis have a less favorable outcome. Fibrous Histiocytoma Dermatofibroma Sclerosing Hemangioma Fibroxanthoma Nodular Subepidermal Fibrosis  Fibrous Histiocytoma Clinical Features: Fibrous histiocytomas are a diverse group of tumors that exhibit both fibroblastic and histiocytic differentiation. They can occur anywhere in the body but those of the skin are most common and in this location are called dermatofibromas. They are uncommon in the oral/perioral region, with the buccal mucosa being the most common intraoral site. Fibrous Histiocytoma Clinical Features Cont.: Most oral tumors are seen in middle-age and older adults. They typically appear as painless nodular masses and can range in size, with the deep tumors being larger. Local surgical excision is the treatment of choice and recurrence is uncommon. Fibrous Histiocytoma Fibrous Histiocytoma Lipoma Lipoma: Clinical Features Etiology: Benign neoplasm of fat cells; some are developmental. Most common soft tissue tumor in the body, but not so common in the mouth. 38th most common mucosal lesion in adults. Prevalence = 3/10,000.  Lipoma Clinical features: Lipomas appear as asymptomatic, slow-growing, well-circumscribed, yellow to yellow-white benign neoplasms of fat. While common elsewhere in the body, lipomas are uncommon intraorally. The cause of lipomas is unknown. Affect both sexes equally. Age: Middle-aged. Location: Buccal, vestibule. Signs & symptoms: Painless, sessile, yellowish, soft mass. Lipoma Histopathological Features: Mature adipocytes with collagen trabeculae. May or may not be encapsulated. May “infiltrate” great distances into surrounding stroma Slowly enlarge, usually remain less than 3 cm. No malignant transformation. Lipoma Treatment and Prognosis: Excision is the treatment of choice for the oral lipoma. Oral lipomas seem to have a limited growth potential and recurrence is not expected after removal except the infiltrating types.  Leiomyoma Benign tumour of smooth muscles eg uterus, GIT. Rare in the oral cavity. Originate from vascular smooth muscles. Three types: 1- Solid leiomyomas. 2-Vascular leiomyomas (angiomyomas or angioleiomyomas). 3-Epithelioid leiomyomas (leiomyoblastoma). Oral leiomyomas are either solid or vascular ( account for nearly 75% of oral cases) type. Leiomyoma Clinical Features: Occur at any age. Slow-growing, firm, mucosal nodule. Most lesions are asymptomatic, occasionally painful. Solid leiomyomas are normal in colour while angiomyomas exhibit bluish hue. Most common sites are: 1-Lips, tongue, palate and cheeks. 2- Intraoral lesions extremely rare. Present as unilocular radioleucencies of the jaws. Leiomyoma Histopathology: Solid types are well-circumscribed tumours consist of interlacing bundles of spindles-shaped smooth muscle cells. Nuclei are elongate, pale staining and blunt ended. Mitotic figers uncommon. Angiomyomas also well circumscribed and demonstrate multiple tortuous blood vessels with thickened walls due to hyperplasia of smooth muscle coat. Intertwining bundles of smooth muscle may be seen between the vessels. Epithelioid type composed of epithelioid cells. Leiomyoma Treatment and Prognosis: Local surgical excision. No recurrence.  Rhabdomyoma Benign tumour of skeletal muscles. Rare tumours. Predilection for the head and neck. Sub classified into: 1-Adult rhabdomyomas. 2- Foetal rhabdomyomas. Rhabdomyoma: Clinical Features Adult Rhabdomyoma: -Middle aged and older patient. -70% of cases found in men. -Most frequent sites are pharynx, oral cavity, larynx. -Intraoral lesions common in the floor of the mouth, base of the tongue. - The tumour appears as a nodule or mass that can grow to many centimetres before discovery. -The laryngeal and pharyngeal types may lead to airway obstruction. - Sometimes the tumour is multinodular or multicentric. Rhabdomyoma: Clinical Features Foetal Rhabdomyoma: -Occur in young children. -May develop in adults. -70% of cases found in men. -Most common in the face and periauricular region. Histopathological Features Adult Rhabdomyoma: Well-circumscribed lobules of large, polygonal cells with abundant granular, eosinophilic cytoplasm. These cells demonstrate peripheral vacuolization that result in the spider web appearance of the cytoplasm. Histopathological Features Foetal Rhabdomyoma: Has less mature appearance. Consist of a haphazard arrangement of spindle-shaped muscle cells within myxoid stroma. Some tumours may show considerable cellularity and mild pleomorphism may be mistaken for rhabdomyosarcoma. Rhabdomyoma Treatment and Prognosis: Local surgical excision. Recurrence has not been reported. Traumatic Neuroma Amputation Neuroma Traumatic Neuroma (Amputation Neuroma) This lesion is a reactive proliferation of neural tissue following transection or damage to the nerve bundle. Traumatic Neuroma: Clinical Features These lesions generally present as smooth, non-ulcerated nodules. Predilection sites include: mental foramen, tongue and lip. There is often a history of trauma. They may produce a radiolucent defect if bone is involved. They may occur at any age but most cases occur in middle-aged adults and there is a slight female gender predilection. Only 25-33% are reported to be painful. If painful, the pain can be constant or intermittent and mild to severe. Lesions involving the mental foramen are most often painful especially if there is contact by a denture. Traumatic Neuroma: Histological Features Presents as a haphazard proliferation of mature, myelinated nerve bundles within a fibrous connective tissue stroma. The lesion may have an associated chronic inflammatory cell infiltrate. Traumatic Neuroma Traumatic Neuroma  Traumatic Neuroma Treatment and Prognosis: Surgical excision is the treatment of choice with the excision to include a small portion of the involved nerve bundle. Most lesions do not recur. Palisaded Encapsulated Neuroma  Palisaded Encapsulated Neuroma Clinical Features: The etiology of this lesion is unknown but trauma has been suggested as a cause. The lesion usually appears as a solitary, smooth, painless, dome-shaped papule or nodule. It occurs more commonly in adults and there is no gender predilection. Common sites include the face, palate and lip.  Palisaded Encapsulated Neuroma Histological Features: As the name suggests the lesion is typically well-circumscribed and often encapsulated. The tumor consists of interlacing fascicles of spindle cells, which are probably Schwann cells. The nuclei are wavy and pointed. While there is palisading there are no Verocay bodies of Antoni A tissue. Histological Features Palisaded Encapsulated Neuroma PEN VS Schwannoma  Palisaded Encapsulated Neuroma Treatment and Prognosis: Treatment consists of conservative local excision and recurrence is rare. This lesions is not associated with neurofibromatosis, multiple endocrine neoplasia syndrome and it does not undergo malignant change. Neurilemoma (Schwannoma)  Neurilemoma (Schwannoma) This lesion is a benign neural neoplasm of Schwann cell origin. It is a relatively uncommon lesion, although 25-48 % of all cases occur in the head and neck region.  Neurilemoma Clinical Features: The neurilemoma is a slow-growing, encapsulated tumor associated with the nerve trunk.. It is usually asymptomatic but pain may occur. Most lesions occur in young to middle-aged adults. The tongue is the most common oral site. The lesion may occur in bone where it may cause expansion, radiolucency, pain or paresthesia.  Neurilemoma Histological Features: The neurilemoma is an encapsulated tumor composed of varying amounts of Antoni A tissue and Antoni B tissue. Antoni A tissue appears as streaming fascicles of spindle-shaped Schwann cells. These cells often form a palisaded arrangement around an acellular eosinophilic area known as a Verocay body.  Neurilemoma Histological Features Cont.: Verocay bodies represent reduplicated basement membrane and cytoplasmic processes.. Antoni B tissue is less cellular and less well organized. Neurites can not be demonstrated within the mass. Ancient neurilemomas(degenerative changes) consist of hemorrhage, hemosiderin deposits, inflammation, fibrosis, and nuclear atypia. May be mistaken for sarcoma. Neurilemoma Treatment and Prognosis: Surgical excision is the treatment of choice and the lesion should not recur. Extremely rare malignant transformation. Neurofibroma/Neuro- fibromatosis  Neurofibroma/Neurofibromatosis Cause: Unknown for the solitary neurofibromas while neurofibromatosis is an autosomal dominant disease entity. Approximately 50 % of cases of neurofibromatosis present no family history and are considered the result of spontaneous mutation. Neurofibromatosis is associated with NF1 and NF2 genes.  Neurofibroma/Neuro-fibromatosis Clinical Features: These lesions are soft, single or multiple, asymptomatic nodules covered by epithelium. Intraorally, they may appear as the same color as or lighter in color than the surrounding mucosa. Most frequently they are found on the tongue, buccal mucosa and vestibule but may occur anywhere. They may occur at any age and there is no gender predilection.  Neurofibroma/Neuro-fibromatosis Histological Features: Neurofibromas show considerable variation. Consist of Schwan cells and fibroblasts with variable amount of collagen and mucoid ground substance. Few nerve fibres run through the lesion. Plexiform neurofibromas are characteristic of neurofibromatosis. They arise around or within a nerve trunk and consist of convoluted nerves surrounded by proliferation of Schwann cells and fibroblasts.  Neurofibroma/Neurofibromatosis Treatment and Prognosis: The treatment of solitary neurofibromas is excision and recurrence is not expected. Multiple neurofibromas should suggest neurofibromatosis (Von Recklinghausen disease), which consists of: -Multiple neurofibromas with malignant potential. -Café au lait spots. -Optic gliomas. -Lisch nodules (iris hamartomas). - Bony lesions as outlined in the next slide.  Box 12-1 Diagnostic Criteria for Neurofibromatosis Type I The diagnostic criteria are met if a patient has two or more of the following features: 1. Six or more café au lait macules over 5 mm in greatest diameter in prepubertal persons and over 15 mm in greatest diameter in postpubertal persons. 2. Two or more neurofibromas of any type or one plexiform neurofibroma. 3. Freckling in the axillary or inguinal regions. 4. Optic glioma.  Box 12-1 Diagnostic Criteria for Neurofibromatosis Type I The diagnostic criteria are met if a patient has two or more of the following features cont. 5. Two or more Lisch nodules (iris hamartomas). 6. A distinctive osseous lesion such as sphenoid dysplasia or thinning of long bone cortex with or without pseudoarthrosis. 7. A first-degree relative (parent, sibling, or offspring) with neurofibromatosis type I, based on the above criteria. Multiple Endocrine Neoplasia Type 2B (MEN Type 2B or III) Multiple Endocrine Neoplasia Type III Multiple Mucosal Neuroma Syndrome  Multiple Endocrine Neoplasia Type 2B (MEN Type 2B or III) Cause: The exact cause of MEN 2B is unknown although it has an autosomal dominant pattern of inheritance.  Multiple Endocrine Neoplasia Type 2B (MEN Type 2B or III) Clinical Features: Multiple neuromas may be associated with this syndrome and are more common on the lips, tongue and buccal mucosa. These neuromas are usually the first sign of the condition. The oral neuromas present as soft, painless papules or nodules. Patients usually have a marfanoid body build, characterized by: Thin, elongated limbs with muscle wasting.  Multiple Endocrine Neoplasia Type 2B (MEN Type 2B or III) Clinical Features Cont.: While the face is typically narrow, the lips are usually thick because of the proliferation of nerve bundles. Of greatest significance is the development of medullary carcinoma of the thyroid. These patients may also develop a pheochromocytoma and an associated hypertensive problem.  Multiple Endocrine Neoplasia Type 2B (MEN Type 2B or III) Treatment: While the oral neuromas may be excised, treatment focuses on the prevention of the medullary carcinoma of the thyroid and the observation of the patient for the development of the pheochromocytoma and its accompanying hypertension.  Multiple Endocrine Neoplasia Type 2B (MEN Type 2B or III) Significance: Given the serious nature of the medullary thyroid carcinoma, some investigators advocate prophylactic removal of the thyroid gland at an early age as the cancer is almost certain to occur. The hypertension associated with the development pheochromocytoma may be life-threatening. Mucosal Neuroma of MEN III Neural Tumors: Comparative Features