Abstract
A rare case of a primary intracranial germ cell tumor which was suspected of transformation into rhabdomyosarcoma is reported. A 19-year-old man presented with diabetes insipidus, disturbance of consciousness, homonymous hemianopsia, and hemiparesis. CAT scanning and MR imaging showed a tumor with contrast enhancement in the hypothalamic area. The HCG level was elevated in both the serum and CSF. With a clinical diagnosis of germ cell tumor, radiation therapy combined with the PVB (cisplatin, vincristine, and bleomycin) chemotherapy regimen was implemented, which resulted in complete disappearance of the tumor. The HCG level was also normalized. However, a couple of weeks later, CAT scanning revealed a new lesion in the right occipital area, although there was no reelevation of the HCG level. The patient was readmitted to our institution and treated with steroids and glyceol, since radiation injury was suspected. Some improvement was seen, but then the patient gradually deteriorated, became bedridden, and developed cortical blindness and hemiparesis, followed by paraplegia, which suggested spinal dissemination. He died one and one-half years after the original admission. At autopsy, the primary site around the third ventricle failed to show any residual tumor cells. There was also no sign of tumor invasion of the occipital lobe. However, there were extensive disseminated spinal lesions involving the spinal cord below the mid-thoracic level which were histologically diagnosed as rhabdomyosarcoma.
