title: [Management of hematologic systemic diseases and rare tumor entities with manifestations in the oromaxillofacial area].
abstract: Malignant lymphomas, hematological malignancies, sarcomas, occult head and neck primaries, Merkel cell carcinomas and malignant melanomas are among the tumors that are rarely seen in the head and neck region. Almost 20% of patients with acute leukemia initially present with symptoms of the oral cavity (ulcerations, gingival hypertrophy, etc.). If a malignant lymphoma is suspected, the lymph node should be removed in toto to ascertain diagnosis. Furthermore, in order to make sure that the immunohistological work-up or electron microscopic analysis is adequate, the pathologist should be informed prior to extirpation of the suspicious lymph node. The same diagnostic procedure is indicated for metastases from undifferentiated or small cell cancer of an unknown primary. Metastatic squamous cell or undifferentiated carcinoma to a solitary cervical lymph node from an unknown primary can be cured by multimodal therapy (extirpation, radical neck dissection and adjuvant radiation) in 30% of the cases. Following polychemotherapy, long-term survival may also be achieved in disseminated stages of undifferentiated carcinoma or poorly differentiated adenocarcinoma in an occult primary. When osteosarcoma of the jaw is suspected, core biopsy has to be planned carefully: in order to prevent tumor seeding, the needle track has to be excised during definitive surgery. Most authors propose (neo-)adjuvant chemotherapy (or chemoradiation) for head and neck osteosarcoma, especially when additional risk factors, i.e. a large primary or poorly differentiated sarcoma, are present. Patients with positive margins should receive adjuvant radiotherapy in soft tissue sarcoma. Local control of angiosarcoma is possible exclusively by radiation. Adjuvant radiation is also indicated in Merkel cell carcinoma. Because this tumor spreads in a "cascade" fashion, elective node dissection may also provide a chance for cure. Excision with wide margins is the principal therapeutic step in malignant melanoma. Due to the anatomic localization, adequate resection may not be possible in mucosal melanoma of the head and neck. When regional lymph nodes are involved, radical lymph node dissection and adjuvant radiation have to be added to the therapeutic concept. There is an emerging role for adjuvant interferon alpha in intermediate and high-risk melanoma.
authors.affiliation: Medizinische Universitätsklinik, Knappschaftskrankenhaus Bochum.
authors.forename: S
authors.initials: S
authors.lastname: Petrasch
mesh_terms: D003131:Combined Modality Therapy; D006258:Head and Neck Neoplasms / Q000556:secondary / Q000628:therapy*; D019337:Hematologic Neoplasms / Q000628:therapy*; D006801:Humans; D007938:Leukemia / Q000628:therapy*; D008223:Lymphoma / Q000628:therapy*; D009382:Neoplasms, Unknown Primary / Q000628:therapy; D010257:Paraneoplastic Syndromes / Q000628:therapy*; D011379:Prognosis