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Sacrococcygeal germ cell tumors

Authors
Rachel A Egler, MD
Alberto S Pappo, MD
Deborah Levine, MD
Louise Wilkins-Haug, MD, PhD
Section Editors
Patrick Y Wen, MD
Jay S Loeffler, MD
Charles J Lockwood, MD, MHCM
Deputy Editors
April F Eichler, MD, MPH
Vanessa A Barss, MD, FACOG

INTRODUCTION

Germ cell tumors (GCTs) that arise outside the testes or the ovaries are classified as extragonadal. 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, sacrococcygeal teratomas (SCTs) are most common.

SCTs are discussed here. Extragonadal GCTs arising in the central nervous system and in the mediastinum and retroperitoneum are discussed elsewhere. (See "Intracranial germ cell tumors" and "Extragonadal germ cell tumors involving the mediastinum and retroperitoneum".)

EPIDEMIOLOGY

SCT is the most common GCT of childhood. In the pediatric population, SCTs account for 40 percent of all GCTs and up to 78 percent of all extragonadal GCTs. Rarely, sacrococcygeal teratomas may present in adulthood [1,2].

SCT is the most frequently recognized fetal neoplasm, with an estimated incidence of about 1 in 27,000 [3]. SCT are more common in females than males, with a 3 to 4:1 ratio [4,5]. SCTs with malignant elements generally are not seen in infants. The incidence of malignant elements within SCTs increases with age.

PATHOLOGY

Histology — Teratomas often are comprised of cells that represent all three germ cell layers. They have solid, cystic or mixed components. Unlike teratomas in other locations, SCTs often do not have a capsule or pseudocapsule, which contributes to the difficulty in achieving a complete resection.

              

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Literature review current through: Nov 2016. | This topic last updated: Fri Mar 25 00:00:00 GMT 2016.
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