Successful multidisciplinary treatment of an endodermal sinus tumor of the pineal region.

The case presented is a 17-year-old male patient with a pineal region endodermal sinus tumor manifested by obstructive hydrocephalus and upward gaze limitation. The highly aggressive nature of this rare malignancy was evidenced by spontaneous tumor bleeding. The patient was treated with a cerebrospinal fluid (CSF) shunt and microsurgical tumor excision followed by irradiation and combination chemotherapy consisting of cisplatin, vincristine, methotrexate and bleomycin. The response to therapy was monitored by serial measurements of serum and CSF alpha-fetoprotein level. The patient remains free of any clinical or radiographic evidence of tumor recurrence 38 months after the initial diagnosis. This experience supports the rationale for an aggressive multidisciplinary approach toward this rare and highly malignant neoplasm.