Clin Pediatr Hematol Oncol.  2016 Apr;23(1):17-27. 10.15264/cpho.2016.23.1.17.

Current Trends in Management for Central Nervous System Germ Cell Tumor

Affiliations
  • 1Division of Pediatric Hemato-Oncology, Department of Pediatrics, Yonsei University College of Medicine, Yonsei University Health System, Seoul, Korea.
  • 2Department of Pediatric Hemato-Oncology, Yonsei Cancer Center, Yonsei University Health System, Seoul, Korea.
  • 3Division of Pediatric Hematology/Oncology, Department of Pediatrics, University of Ulsan College of Medicine & Asan Medical Center, Seoul, Korea.
  • 4Department of Pediatrics, Kyungpook National University Hospital, Kyungpook National University School of Medicine, Daegu, Korea.
  • 5Department of Pediatrics, Chonnam National University Medical School, Chonnam National University Hwasun Hospital, Gwangju, Korea.
  • 6Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
  • 7Department of Pediatric Neurosurgery, Severance Hospital, Yonsei University Health System, Seoul, Korea. dskim33@yuhs.ac
  • 8Department of Radiation Oncology, Yonsei Cancer Center, Yonsei University Health System, Seoul, Korea.

Abstract

Central nervous system germ cell tumor is a rare but important tumor in childhood brain tumors. It requires a multidisciplinary approach to increase survival and promote quality of life, and all three treatment modalities including surgery, radiotherapy and chemotherapy has its own distinct role for germ cell tumor. For germinoma, radiotherapy alone can cure the disease but, the effort to limit the long term toxicity and the proper combination of chemotherapy and radiotherapy are under investigation. Craniospinal irradiation is reserved only for the disseminated germinoma or nongerminomatous germ cell tumor (NGGCT). For germinoma, craniospinal irradiation of 20 to 24 Gy is sufficient to control microscopic disease in the spinal axis. Chemotherapy and radiotherapy composed of 30 to 40 Gy of local field radiotherapy and 20 to 24 Gy of whole ventricular irradiation are required for localized germinoma, but the proper combination of two modalities has yet to be defined. For NGGCT, both the chemotherapy and radiotherapy should be performed, and survival rate is substantially increasing with modern treatment protocols. The omission of craniospinal irradiation is being tried for the localized NGGCT in international cooperative group trials. Surgery has its role for the resection of residual disease after the treatment, and the extent of resection in NGGCT has the prognostic implication. Bifocal germ cell tumors and basal ganglia germ cell tumor have distinctive clinical course and mandate special attention. To advance clinical and biological perspectives in central nervous germ cell tumor, the cooperation and communication of the multidisciplinary specialists are essential.

Keyword

Neoplasms; Germ cell and embryonal; Brain neoplasms; Germinoma; Neoplasms

MeSH Terms

Axis, Cervical Vertebra
Basal Ganglia
Brain Neoplasms
Central Nervous System*
Clinical Protocols
Craniospinal Irradiation
Drug Therapy
Germ Cells*
Germinoma
Neoplasms, Germ Cell and Embryonal*
Quality of Life
Radiotherapy
Specialization
Survival Rate
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