324

PDQ ORAL DISEASE

Malignant Nonodontogenic Tumors

Ewings Sarcoma
Etiology Unknown Chromosomal translocations t(11;22), t(7;22), t(7;21) noted Gene rearrangement often noted, that is, (22;q12) and expression of the MIC2 gene Genetically related to primitive peripheral neuroectodermal tumor via translocations t(11;22), (q24;q12) Clinical Presentation 60% in males; over 95% in those under 20 years of age Chiefly in bone and soft tissues Highly malignant Pain, numbness, and swelling often early complaints Diffuse, irregular, lytic bone lesion Cortical expansion variable Second most common bone tumor of children/adolescents Soft tissues of head and neck account for 11% of extraskeletal sites Diagnosis Radiographs often show moth-eaten appearance and laminar periosteal bone reaction Cortex may be eroded or expanded Differential Diagnosis Osteosarcoma Lymphoma Peripheral neuroectodermal tumor of bone Primitive rhabdomyosarcoma Neuroectodermal tumor of infancy

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Treatment Radiation and multiagent chemotherapy Prognosis 54 to 74% 5-year survival rate in localized osseous form Late relapse not uncommon

326

PDQ ORAL DISEASE

Metastatic Cancer
Etiology Spread of a primary malignancy to the oral cavity structures or jaws (usually from lung, breast, prostate, colon, kidney) Accounts for < 1% of oral malignancies Clinical Presentation Usually manifests in the jaws with pain and swelling Not uncommon is loosening of teeth or pathologic jaw fracture Soft tissue location is rare. Most frequent sites of primary neoplasms are kidney, lung, breast, colon, prostate, stomach Intraosseous lesions with lytic, ill-defined radiolucencies Microscopic Findings As with the primary tumor Tumor marker studies (immunohistochemical) may be necessary to define the site of origin. Diagnosis Radiographic findings Biopsy Differential Diagnosis Primary soft tissue tumor Primary osseous tumor Periodontitis (localized) Osteoradionecrosis Treatment Local radiation Combination chemoradiotherapy Prognosis Poor

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328

PDQ ORAL DISEASE

Osteosarcoma
Etiology May be associated with pre-existing bone disease such as the following: Pagets disease (10 to 15%) Fibrous dysplasia (0.5%) Mutation/amplification of p53, c-myc, c-JUN, c-fos, MOM2, CDK4, SAS Clinical Presentation May present with pain paresthesia, trismus, nasal or paranasal sinus obstruction May masquerade as an odontogenic infection Intraoral signs are as follows: Tooth mobility (vertical) Periapical radiolucency (teeth vital) Distal displacement of terminal molar Jaw mass may be ulcerated. Radiographic Findings Early intraoral findings Displacement of teeth Root resorption Absent or attenuated lamina dura Uniformly widened periodontal membrane space Later jaw bone findings Lytic, moth-eaten destruction Cortical destruction Soft tissue extension Erosion of mandibular canal 25% of cases have sunburst effect (radiating radiopaque spicules) Microscopic Findings Sarcomatous stroma Osteoid production by neoplastic cells Four basic patterns (no prognostic significance) are as follows: Osteoblastic Fibroblastic Chondroblastic Telangiectatic

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Diagnosis Correlation of clinical, radiographic, pathologic findings Differential Diagnosis Fibro-osseous lesion Osteomyelitis Osteoradionecrosis Metastatic tumor Other form of sarcoma Treatment Radical ablative surgery Hemimandibulectomy Partial maxillectomy orbital exenteration Adjuvant chemotherapy/radiotherapy Prognosis Survival ranges from 12 to 58% at 5 years Mandibular lesions are associated with a greater survival rate than are maxillary lesions.