A Case of Atypical Teratoid/Rhabdoid Tumor Arising from the Supratentorial Area / 소아과

Atypical teratoid/rhabdoid tumor may arise at any central nervous system location, but it is most commonly located in the cerebellum(60 percent). The incidence of this tumor remains unclear but it occurs most commonly in children less than 2 years of age. This highly malignant tumor shows a rapid progression and nonspecific radiologic findings. We report a case of primary intracranial atypical teratoid/rhabdoid tumor arising from the supratentorial area in early infancy. The diagnosis was made based on distinctive light microscopy and immunohistochemical findings. Despite aggressive surgical treatment with adjuvant chemotherapy, he died six months after his second operation.

A Clinical Analysis of Pediatric Brain Tumors

The authors analyzed 50 children(below 15 years old) with intracranial tumors which were confirmed by operation during 5.5 years from Jan, 1983 to Jul. 1989 retrospectively. The results were as follows : 1) Incidence of pediatric brain tumors was 13.1% among the all intracranial tumors(383 cases). 2) Pediatric brain tumors were found most frequently between the age of six and thirteen years(74.0%), and the ratio of male to female was 1.6 : 1. 3) In respect of location, supratentorial and infratentorial tumors were seen in each 25 patients, and the tumor of central neural axis(58.0%) was more frequent than that of lateral portion(42.0%). 4) The most common tumor was astrocytoma(32.0%) in the supratentorium, and medulloblastoma(26.0%) in the infratentorium. 5) Headache and vomiting were the most common complaining symptoms and papilledema was the most common sign. 6) Hydrocephalus associated with tumors was 24.0% in supratentorium and 88.0% in infratentorium. 7) Postoperative mortality within 1 month was 12.0%.