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Intracranial germ cell tumors

Author
Jack M Su, MD, MS
Section Editors
Jay S Loeffler, MD
Patrick Y Wen, MD
Amar Gajjar, MD
Deputy Editor
April F Eichler, MD, MPH

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. Throughout the text we generally do not distinguish between children and adults. It is important to note, however, that the literature on intracranial GCTs is largely based on children under the age of 15 years, and historical data and outcomes are being extrapolated to young adults based on a small proportion of these patients in most series.

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 0.5 to 3 percent of pediatric central nervous system (CNS) tumors [1]. In contrast, these tumors are substantially more frequent in Japan and other Asian countries, with a reported incidence of up to 11 percent of pediatric CNS tumors [1]. Even in the United States, individuals with Asian/Pacific Islander ancestry have a two- to threefold higher risk of intracranial GCT compared with whites, suggesting that genetic factors may be more important than environmental factors in the etiology of GCT [2].

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 [3,4].

                               

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Literature review current through: Nov 2016. | This topic last updated: Fri Apr 01 00:00:00 GMT+00:00 2016.
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