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[Askin tumor. Rare neurogenic tumor with metastasis to mouth, jaw and face regions].

One case of a malignant peripheral neuroectodermal tumor of the thoracopulmonary region (Askin-Tumor) is reported. The tumor represents a distinct pathologic entity of neuroectodermal origin. The patient developed a metastasis in the upper jaw and in a lumbar vertebra. Morphologic and immunocytochemical findings of this extremely rare tumor were described and differential diagnosis from other soft-tissue round-cell sarcomas, such as Ewing's sarcoma and neuroblastoma are discussed. Problems associated with the clinical behavior and treatment are outlined. The prognosis in most cases of Askin-tumor is poor. The recommended therapy is radical resection, irradiation and combination chemotherapy containing anthracyclines and a high dose of alkylating agents.