From 1962 through 1987, four children were diagnosed at our institution with primary germ cell malignancies of the extracranial head and neck regions. Ages of the children ranged from 2 to 44 months. Histologic findings included 2 yolk sac carcinoma (endodermal sinus tumor), 1 malignant teratoma with neuroblastoma (primitive neuroectodermal) components. Complete clinical and surgical staging was performed to rule out additional sites of disease. All patients initially underwent either biopsy or, when technically feasible, resection. Three patients received combination chemotherapy and two received irradiation. Three patients died of progressive disease. One patient who had yolk sac carcinoma of the temporomandibular region is alive and free of disease 40 months after therapy. Complete surgical resection is indicated for teratomatous tumors, if technically feasible. The malignant components of these tumors are sensitive to both chemotherapy and irradiation and combined therapy may be beneficial.
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