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The Oncologist, Vol. 13, No. 6, 690-699, June 2008; doi:10.1634/theoncologist.2008-0037
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 Pediatric Central Nervous System Germ Cell Tumors: A Review
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Pediatric Oncology

Pediatric Central Nervous System Germ Cell Tumors: A Review

Maria E. Echevarría, Jason Fangusaro, Stewart Goldman

Division of Neuro-Oncology, Department of Pediatric Hematology-Oncology and Stem Cell Transplantation, Children's Memorial Hospital, Chicago, Illinois, USA

Key Words. Neoplasms • Germ cell and embryonal • Brain neoplasms • Pediatrics

Correspondence: María E. Echevarría, M.D., 2300 Children's Plaza Box #30, Chicago, Illinois, 60614, USA. Telephone: 773-880-4562; Fax: 773-880-3223; e-mail: maleneche{at}gmail.com

Received February 14, 2008; accepted for publication April 27, 2008.

Disclosure: No potential conflicts of interest were reported by the authors, planners, reviewers, or staff managers of this article.

Central nervous system (CNS) germ cell tumors (GCTs) represent approximately 3% of primary pediatric brain tumors and encompass a wide pathologic spectrum. CNS GCTs are most commonly located in the pineal and suprasellar regions of the brain and can be divided into major groups including germinomas and nongerminomatous GCTs (NGGCTs), with teratomas often considered a separate category. The clinical presentation varies by location and size, and it frequently includes endocrine abnormalities, visual changes, and signs of increased intracranial pressure. Neuroimaging studies cannot differentiate GCTs from other tumors, and therefore, the diagnosis usually requires histologic confirmation. The rare exceptions are the cases where characteristic elevations of tumor markers, including alpha-fetoprotein and/or β-human chorionic gonadotropin are documented in the serum and/or cerebrospinal fluid. In these cases, the imaging findings along with the tumor marker elevation may be diagnostic in themselves without the need for tissue confirmation.

Treatment and prognosis differ greatly between groups. Germinomas have a superior prognosis than NGGCTs. Five-year overall survival rates >90% were reported initially with the use of craniospinal irradiation. More recently, the use of chemotherapy in addition to radiation therapy has afforded the ability to decrease the dose and volume of radiation therapy without affecting survival rates. NGGCTs are less radiosensitive than germinomas, but the use of adjuvant chemotherapy has improved survival rates in this group as well. The standard management for CNS GCTs remains controversial. Treatment regimens aimed to improve progression-free and overall survival times are ongoing.






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