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Pediatric intracranial germ cell tumors

INTRODUCTION

Germ cell tumors (GCTs) are classified as extragonadal if there is no evidence of a primary tumor in either the testes or the ovaries. Extragonadal GCTs typically arise in midline locations, and specific sites vary with age. In adults, the most common sites are the anterior mediastinum, retroperitoneum, and the pineal and suprasellar regions. In infants and young children, intracranial GCTs and sacrococcygeal teratomas are more common than other locations.

Intracranial GCTs are discussed here. Sacrococcygeal teratomas and extragonadal GCTs arising in the mediastinum and retroperitoneum are discussed elsewhere. (See "Sacrococcygeal germ cell tumors" and "Extragonadal germ cell tumors involving the mediastinum and retroperitoneum".)

EPIDEMIOLOGY

In North America and Europe, intracranial GCTs represent 1 to 2 percent of pediatric central nervous system (CNS) tumors [1,2]. In contrast, these tumors are substantially more frequent in Japan and other Asian countries, with a reported incidence of 5 to 12 percent [2-4].

The peak incidence of intracranial GCT is during the second decade of life, with a median age at diagnosis of 10 to 12 years. There is a male preponderance of between 2:1 to 3:1, especially with tumors in the pineal region [1,2].

ETIOLOGY

Histogenesis — Various theories have been proposed to explain the origin of extragonadal GCTs. These are discussed elsewhere. (See "Extragonadal germ cell tumors involving the mediastinum and retroperitoneum", section on 'Histogenesis'.)
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