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Oral Pathology
EPULIS FISSURATUM
Synonyms: Inflammatory fibrous hyperplasia, denture injury tumor & denture epulis Is tumor-like hyperplasia of fibrous connective tissue associated with the flange of an ill-fitting denture Epulis: any "tumor" of gingiva or alveolar mucosa Typically presents; as single or multiple folds of hyperplastic tissue in alveolar vestibule Most often are two folds with denture flange fitting between the two Usually firm & fibrous but may show erythema and ulceration Usually on facial aspect; size varies Clinical Features: Most common in middle-aged /older adults More common in anterior of either jaw Female predilection; uncertain as to why but maybe: more older women wear dentures women live longer, more women go to , women wear their dentures more hours/ day postmenopausal. hormonal chances Histologic Features: Hyperplasia of fibrous connective tissue Overlying epithelium often shows hyperparakeratosis & irregular rete ridges Inflammatory papillary hyperplasia or PEH Ulceration is not unusual and a chronic inflammatory infiltrate is pest Rarely osteoid or chondroid may be seen Treatment & Prognosis: Treatment consists of surgical removal with microscopic examination of the tissue
FIBROUS HISTIOCYTOMA
Diverse group of tumors with bode fibroblastic & histiocytic-differentiation Cell of origin is uncertain Clinical Features: Most common site is skin of extremities Uncommon in oral region If oral, then most common sites are buccal mucosa and vestibule Patient are middle-aged or older adults Appears as painless nodular mass with varying size Histologic Features: Characterized by cellular proliferation of spindle-shaped fibroblastic cells with vesicular nuclei Storiform pattern Round histiocyte-like cells, xanthoma cells & multinucleated giant cells may be preset along with scattered lymphocytes Treatment & Prognosis: Local surgical excision Recurrence is uncommon but higher in larger, deep lesions
Cells at periphery often have marked eosinophilia reminiscent of smooth muscle Treatment & Prognosis: Fibromatosis is treated by wide surgical excision because it is-locally aggressive Oral/paraoral fibromatosis has approximately a 23% recurrence rate Myofibromatosis can be treated by local excision; spontaneous regression may. occur Multicentric lesions: less favorable outcome
PYOGENIC GRANULOMA
Clinical Features: Asymptomatic red tumescence composed of granulation tissue; most commonly seen on gingiva; can be seen anywhere on skin or mucosa; periodontal ligament origin; may be secondarily ulcerated; common Cause: Trauma or chronic irritation; size modified by hormonal changes Treatment: Excision Significance: Remains indefinitely; recurrence if incompletely excised; reduction in size if cause removed or after pregnancy
Color: red to pink & surface often ulcerated Young adults and more common in females Slight predilection for maxillas & anterior Histologic Features: Fibrous proliferation associated with mineralized product Epithelium may show ulceration with fibrinopurulent membrane Often cellular around mineralized areas: mineral may be bone, cementum-like or dystrophic calcification Treatment & Prognosis: Local surgical excision down to periosteum to prevent recurrence Adjacent teeth should be scaled to eliminate irritants A recurrence rate of 16% has been reported
LIPOMA
Clinical Features: Asymptomatic, slow-growing, well-circumscribed, yellow or yellow-white tumescence; rare benign neoplasm of fat; occurs in any area Cause: Unknown Treatment: Excision Significance: Seems to have limited growth potential intraorally Recurrence not expected after removal
Palisading but no Verocay bodies of Antoni A tissue Treatment & Prognosis: Treatment consists of conservative local excision Recurrence is rare Lesion is not associated with neurofibromatosis, multiple endocrine neoplasia syndrome and no malignant-change
NEURILEMOMA (SCHWANNOMA)
Benign neural neoplasm of Schwann cell origin Is relatively uncommon although 25-48% of all cases occur in head-and neck area Clinical & Radiographic Features: Slow growing, mated tumor associated with nerve trunk Usually asymptomatic but pain may occur Most common in young/ middle-aged adults Tongue is most common site;. in bone may cause expansion, RLs, pain & paresthesia Histologic Features: Encapsulated tumor with varying amounts of Antoni A and Antoni B tissue Antoni A: streaming fascicles of spindle-shaped Schwann cells; these cells often form a palisaded arrangement around acellular eosinophilic areas known as Verocay bodies Verocay bodies represent reduplicated basement membrane and cytoplasmic processes Antoni B tissue is less cellular & organized Neurites cannot be demonstrated within mass Ancient neurilemomas Treatment & Prognosis: Surgical excision is treatment of choice and the lesion should not recur Extremely rare malignant transformation
NEUROFIBROMA NEUROFIBROMATOSIS
Clinical Features: Soft, single or multiple, asymptomatic nodules covered by epithelium; same as-or lighter than surrounding mucosa; most frequently seen on tongue, buccal mucosa and vestibule but may be seen anywhere; any age; uncommon Cause: Unknown for solitary; neurofibromatosis is autosomal dominant Treatment: Excision Significance: Recurrence not expected; multiple neurofibromas should suggest von Recklinghausen's disease (neurofibromas with malignant potential; cafe au lait spots)
PARAGANGLIOMA
Paraganglia are specialized tissues of neural crest origin associated with autonomic nerves and ganglia throughout the body Tumors of the paraganglia are collectively known as paragangliomas with each taking on a specific name as to the site of development; the most common is the carotid body tumor Histologic Features: Paraganglioma are characterized by round or polygonal epithelioid cells organized into nests or Zellballen Tumors are typically vascular & may be surrounded by a thin capsule Most are benign but malignant behaving ones are difficult to recognize by histology
CONGENITAL EPULIS
Clinical Features: Firm pedunculated or sessile mass attached to gingiva in infants; same color as or lighter than surrounding tissue; rare Cause: Unknown Treatment: Surgical excision; spontaneous regression in some cases Significance: A benign neoplasm of granular cells similar to the granular cell tumor of the adult; does not recur
HEMANGIOMA
Clinical Features: Red/blue lesion, that blanches when compressed; extent of lesion usually difficult to determine; skin, lips, tongue and buccal mucosa most commonly affected; common on skin; uncommon in mucous membrane, rare in bone Cause: Souse are benign, congenital tumors; other, are due to abnormal vessel morphogenesis Treatment: Most congenital lesions involute; vascular malformations may require surgical removal, sclerosing agents, etc. Significance: May remain quiescent or gradually enlarge hemorrhage may be a significant complication; often a cosmetic problem
STURGE-WEBER ANGIOMATOSIS
Rare, non-hereditary developmental condition Hamartomatous vascular proliferation involving face and brain characteristic Clinical Features: Pts. born with capillary vascular malformation of face known as port-wine stain or nevus flammeus Leptomeningeal angiomas overlie ipsilateral cerebral cortex with associated convulsive disorder, mental retardation or contralateral hemiplegia Skull radiographs may show "tram-line" calcifications 6
Ocular involves includes- glaucoma & vascular malformations Intraoral involvement is common & presents as hypervascular charges to mucosa, gingiva Histologic Features: Port-wine nevus is characterized by excessive numbers of dilated blood vessels Oral lesions show a similar appearances Proliferative gingival lesions may resemble pyogenic granulomas Treatment & Prognosis: Treatment and prognosis depends upon seventy of case Port-wine nevi have been removed by laser Symptomatic leptomeningeal lesions are neurosurgical cases Law therapy has also be used for intraoral cases; beware of surgery in affected areas
NASOPHARYNGEAL ANGIOFIBROMA
Rare vascular/ fibrous nooplasm which occurs only in nasopharynx Frequently is locally destructive & aggressive Clinical Features: Almost exclusively in males & a predilection for the young Nasal obstruction & epistaxis are common early symptoms Destructive tumor can extend into adjacent structures; anterior bowing of posterior wall of maxillary sinus is characteristic Histologic Features: Composed of dense fibrous connective tissue containing numerous dilated, thin walled blood vessels The vascular component is often more prominent at the periphery Treatment & Prognosis: Surgical excision is the usual treatment Preoperative embolization can help prevent blood loss Radiation is usually reserved for recurrent lesions Recurrence rate approx. 20%; malignant transformation has rarely been reported
HEMANGIOPERICYTOMA
Clinical Features: Rave neoplasm of pericytes which is seen primarily in adults with no sex predilection Usually a slow growing, painless mass A form of the tumor occurs in nasal/ paranasal sinuses where it presents with obstruction and epistaxis Histologic Features: Fairly well circumscribed mass of tightly packed cells surrounded by endothelium vascular channels Blood vessels show irregular branching: "staghorn"; dense reticulin network surrounds vessels 4+ mitoses / 10 H.P. fields = rapidly growth Treatment & Prognosis: For those with a benign histopathologic appearance, local excision; more extensive surgery required for tumors with malignant characteristics Most tumors are biologically benign however there is a 40-50% local recurrence rate and a 11-56% rate of metastasis overall
LYMPHANGIOMA
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Clinical Features: Spongy, diffuse, painless mass in dermis; may become large; lighter than surrounding tissue to red / blue; crepitance; children; rare Cause: Developmental Treatment: Excision Significance: May be disfiguring or cause respiratory distress
LEIOMYOMA
Benign neoplasms of smooth muscle which are rare in the oral cavity There are 3 types: solid, vascular, and epithelioid; 75% of oral are angiomyomas Clinical Features: Can occur at any age. Usually slow growing firm nodule which-is asymptomatic Solid tumors have a normal color, while vascular ones are usually bluish Lips, palate, tongue, & cheek most common site Histologic Features: Solid type: well-circumscribed tumor of interlacing bundles of spindle-shaped smooth muscle. cells; nuclei are elongate, pale-staining aid blunt-ended Angiomyomas: demonstrate multiple tortuous blood vessels in addition to intertwining bundles of smooth muscle Treatment & Prognosis: Treated by local surgical excision Lesion should not recur
RHABDOMYOMA
Benign neoplasm of skeletal muscle Two categories: adult & fetal Adult: middle-aged/older with male predilection Most common in floor of mouth, soft palate & base of tongue Presents as nodule or mass, solitary or multicentric Fetal: usually in young children but an occasional adult; also a male predilection Histopathologic Features: Adult: well-ciramoscribed lobules of large polygonal cells with abundant granular eosinophilic cytoplasm; cells show peripheral vacuolization giving "spider web" appearance Fetal: less mature with haphazard arrangement of spindle-shaped muscle cells in myxoid stroma; some tumors are quite cellular and have mild pleomorphism Cross-striations are helpful in diagnosis as are special stains Treatment & Prognosis: Local surgical excision is treatment of choice in both types Recurrence is uncommon
Occasionally, both tissues are found together Treatment & Prognosis: Local surgical excision Recurrence has not been reported
FIBROSARCOMA
Clinical Features: Malignant tumor of fibroblasts only 10% of which occur in head & neck region Often slow growth & may reach a large size before being painful Most common sites: nose & paranasal sinuses Any age but mostly children young adults Histologic Feature: Well-differentiated tumors consist of fascicles of spindle shaped cells forming a "herringbone" pattern; cells show little pleomorphism but mitotic figures present Poorly differentiated tumors show less organization, cells more pleomorphic, more mitotic activity and less collagen produced Treatment & Prognosis: Surgical excision with a wide margin is treatment of choice Recurrence rates vary from 20-60% 5-year survival rates range from 40-70%
MALIGNANT FIBROUS-HISTIOCYTOMA
Clinical Features: Malignant tumor with both fibroblastic & histiocytic differentiation Considered most common soft tissue sarcoma in adults; rare in head & neck Most common in older age group Patients complain of enlarging mass which may or may not be painful & ulcerated Histologic Feature: Several histologic subtypes: storiform-pleomorphic most common Treatment / Prognosis: Is aggressive; usually treated by radical surgical excision; approx. 40% have local recurrences & an equal percentage have metastases usually within 2 years; oral tumors lead to particularly- poor survival rates
LIPOSARCOMA
Clinical Features: Malignant tumor of adipose tissue; second most common soft tissue sarcoma in adults Rare in head & neck; cheek must common intraoral site Peak prevalence between 40-60 yrs. Usually soft, slow growing, ill-defined mass; yellow or normal in color; pain or tenderness is uncommon Histologic Feature: 4 histologic subtypes: myxoid liposarcoma accounts for almost 50% of cases Myxoid subtype slows lipoblasts within a myxoid stroma with many capillaries Treatment / Prognosis: Radical excision is treatment of choice 50% recurrence; 10-yr. survival approx. 50%; subtype is important in predicting prognosis 9
NEUROFIBROSARCOMA
Clinical Features: Principal malignancy of peripheral nerve origin; 50% of cases in patients with neurofibromatosis; rare in head & neck Mean age with neurofibromatosis (29 yrs.) without (40 yrs.) Enlarging mass, sometimes with rapid growth, pain; nerve deficit Common sites; mandible, lips & cheek Histologic Features: shows fascicles of atypical spindle-shaped cells which often resemble cells of fibrosarcomas but cells more irregular with wavy or commashaped nuclei; special stains may be helpful. Treatment: consists primarily of radical surgical excision but may also use radiation & chemo. in conjunction; prognosis is poor
OLFACTORY NEUROBLASTOMA
Clinical Features: Rare neuroectodermal neoplasm of upper nasal vault More common in adults Nasal obstruction, epistaxis & pain most common symptoms Histologic Features: small, round-to ovoid basophilic cells arranged in sheets & lobules; rosettes / pseudorosettes may be seen Treatment: Surgical excision often with adjunct radiation therapy; chemotherapy may be added to advanced cases Prognosis: Depends on stage: Stage A: 90% 5 year survival Stage C: 50% 5 year survival Death usually results from local recurrence
ANGIOSARCOMA
Clinical Features: Rare malignancy of vascular endothelium 50% in head & neck region but oral lesions rare Cutaneous angiosarcomas are most common in elderly where they may resemble a bruise Eventually lesion becomes elevated, nodular or ulcerates; may be multifocal; most common oral site: mandible Histologic Features: Characterized by infiltrative proliferation of endothelium-lined blood vessels that form an anastomosing network Cells appear hyperchromatic & atypical & may fill lumen; increased mitotic activity Treatment: consists of radical surgical excision; radiation or both Prognosis: extremely poor
KAPOSI'S SARCOMA
Clinical Features: Pathogenesis is uncertain although multicentricity; geographic distribution & patient population suggests viral origin; CMV has been suggested Sarcoma vs. infectious process 4 clinical presentations: Classic, Endemic (African), Iatrogenic immunosuppression- associated, AIDS-related Classic: Males (90%); older adults; Italian, Jewish or Slavic ancestry; multiple blue/purple macules / plaques on lower extremities; slow growth of painless nodules; oral lesions rare but palate most common oral site; approx. 33% of patients have an associated malignancy 10
Endemic (African) Type: has been divided into 4 subtypes: benign nodular, aggressive, florid,
lymphadenopathic
Iatrogenic Type: most often occurs in recipients of organ transplants; 0.4% of renal transplant
patients
AIDS-related
Histologic Features: Kaposi's typically evolves though 3 stages: patch, plaque, nodular Treatment: depends upon clinical subtype and stage Prognosis: depends upon form of the disease and the patient's immune status
LEIOMYOSARCOMA
Malignant neoplasm of smooth muscle Rare in oral cavity More common in middle-aged & older adults Clinical Features: Clinical appearance is nonspecific i.e.. enlarging mass which may or may not be painful or ulcerated Histologic Features: fasicles of spindle-shaped cells with abundant eosinophilic cytoplasm and bluntended cigar-shaped nuclei; some tumors have round epithelioid cells; pleomorphism and mitoses vary Treatment: Radical surgical excision sometimes with adjunctive chemotherapy or radiation therapy Prognosis: poor
RHABDOMYOSARCOMA
Clinical Features: Malignant neoplasm of skeletal muscle which is the most common soft tissue sarcoma of children; 40% in head / neck area Primarily occurs in first decade; 60% in males; palate most common intraoral site Often presents as a painless, infiltrative mass which may grow rapidly Histologic Features: 3 histologic types: embryonal, alveolar & pleomorphic Most head / neck ones are either embryonal or alveolar Embryonal: most common in first 10 years; 75% cases are embryonal; embryonal resembles various stages in the embryogenesis of skeletal muscle Alveolar: median age is 16 years Treatment: Usually consists of local surgical excision followed by multiagent chemotherapy; postoperative radiation is used except in cases where complete resection has been achieved Prognosis: Overall 5 year survival rate is 63%
SYNOVIAL SARCOMA
Clinical Features: Uncommon tumor that occurs primarily in vicinity of large joints & bursae but most authorities agree it does not arise from the synovium; rare is head &neck region Most common in teens & young adults Slight male predilection Slow enlarged often associated with pain or tenderness In head & neck most tumors are paravertebral or parapharyngeal & often cause dysphagia; dyspnoia or hoarseness Orofacial lesions reported: cheek parotid region & base of tongue Histologic Features: Classic synovial sarcoma is a biphasic tumor composed of spindle and epithelial cells; calcifications seen in 30% of cases Less frequently the tumor is monophasic and consists mostly / entirely of spindle cells Treatment: consists of radical surgical excision possibly combined with radiation or chemotherapy Prognosis: 5 year survival ranges from 36-51% 10 year survival drops to 11-30% because of late metastases
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