CEREBRAL HCG SECRETING TUMER-RESPONSE TO CHEMOTHERAPY

Abstract

A 9-year-old white male with bitemporal vision loss and sexual precocity, had optic atrophy and panhypopituitarism(Peak hGH response to arginine/insulin -3.5ng/ml, cortisol response to hypoglycemia -5μg/ml). The T4 was 1.6ng/100ml and morning urine specific gravity 1.001. His FSH was 5.5mIU/ml but hLF was >100m IU/ml. Tim testosterone was 1, 644ng/100ml. ENI scan was negative. The air study and carotid angiogram revealed a large avascular suprasellar mass. The greatly elevated hLH level and panhypopituitarism suggested hCG of tumor origin which was confirmed at a level of 884ng/ml. The parents refused surgery or radiotherapy on religious grounds. Three months later he was readmitted with signs and symptoms of increased intracranial pressure. Repeat ENI scan revealed a large suprasellar nass with central calcification and obstructive hydrocephalus. A (R) V-A shunt was performed and the patient was started on Methotrexate 3mg/kg every two weeks for six weeks. During this time, he deteriorated to a state of dementia, stupor, blindness, and spasticlty. In the seventh post-op week, triple therapy of Vincrlstine, Actinomycin D, and Cytoxan was initiated. By the 8th week of triple therapy he was a happy, alert, articulate, ambulatory boy. His optic atrophy, and panhypopituitarism persist. By the llth week, the hCG level was <4ng/ml and by the 14th week, his ENI scan revealed a narked reduction in tumor size.