Intracranial growing teratoma syndrome following radiotherapy—an unusually fulminant course

Abstract

Residual radiologically progressive masses following multimodality treatment of malignant mixed intracranial germ cell tumors are described. Often these enlarge, and this is called the growing teratoma syndrome. A similar phenomenon during radiotherapy alone has not been described. A 5-year old boy presented with features of raised intracranial pressure. Imaging revealed a posterior third ventricular mass, which was biopsied endoscopically. A review of the scanty tissue was suggestive of a pineal parenchymal tumor, and hence radiation was planned. After just ten fractions, he developed rapid neurological deterioration. Repeat imaging raised a possibility of a teratomatous tumor. He underwent emergency excision. However, he had a stormy postoperative course and succumbed to deep venous infarction. Histology revealed a purely mature teratoma. Though a growing teratoma syndrome has been described following chemotherapy, no such report while on radiation exists. Ours could be the first such reported case. We discuss the possible mechanisms with a review of the literature.