Central Nervous System Germ Cell Tumor

  • Takamitsu FujimakiEmail author
Part of the Tumors of the Central Nervous System book series (TCNS, volume 4)


Central nervous system germ cell tumors (CNSGCTs) are tumors of children and young adults. They affect mainly the pineal body, neurohypophysis (“suprasellar” area) and basal ganglia, but can arise in any other CNS location. Histologically, they are divided into germinoma and non-germinomatous germ cell tumors (NGGCTs), the latter including teratoma, embryonal carcinoma, choriocarcinoma, and yolk sac tumor. Teratomas are further subclassified into mature teratoma, immature teratoma, and teratoma with malignant transformation. Except for mature teratoma, all of these tumors are biologically malignant. Histologically, they resemble reproductive cells, fetal tissues, or related tissues such as placenta, and mixtures of these tumor subcategories are not rare. Initial symptoms include increased intracranial pressure due to obstructive hydrocephalus, abnormality of ocular movement and endocrine disorders. Some tumors of these subclasses produce humoral tumor markers, beta-human chorionic gonadotropin, alpha-fetoprotein or other markers, which can be used for differential diagnosis or clinical follow-up. Only mature teratoma can be cured with surgery alone, and the other tumors require adjuvant therapy for complete remission. Germinomas respond well to irradiation and can be cured, but platinum-based chemotherapy combined with reduced-dose irradiation has been recently used to minimize radiation-related toxicities or complications. As the other tumors cannot be cured by irradiation alone, more intensive platinum-based chemotherapy and radiation therapy including whole-neuroaxis irradiation are employed. Although better disease control and longer survival can now be achieved, the results of treatment are still not satisfactory. Therefore, development of new treatments, and improvements to existing ones, are needed for CNSGCTs.


CNSGCT Germinoma Teratoma Embryonal carcinoma Choriocarcinoma Yolk sac tumor Teratoma 


  1. Berek K, Aichner F, Schmutzhard E, Kofler M, Langmayr F, Gerstenbrand F (1991) Intracranial germ cell tumor mimicking anorexia nervosa. Klin Wochenschr 69:440–442PubMedCrossRefGoogle Scholar
  2. Calaminus G, Bamberg M, Jurgens H, Kortmann RD., Sorensen N, Wiestler OD, Gobel U (2004) Impact of surgery, chemotherapy and irradiation on long term outcome of intracranial malignant non-germinomatous germ cell tumors: results of the German Cooperative Trial MAKEI 89. Klin Padiatr 216:141–149PubMedCrossRefGoogle Scholar
  3. Eom KY, Kim IH, Park CI, Kim HJ, Kim JH, Kim K, Kim SK, Wang KC, Cho BG, Jung HW, Heo DS, Kang HJ, Shin HY, Ahn HS (2008) Upfront chemotherapy and involved-field radiotherapy results in more relapses than extended radiotherapy for intracranial germinomas: modification in radiotherapy volume might be needed. Int J Radiat Oncol Biol Phys 71:667–671PubMedCrossRefGoogle Scholar
  4. Friedman NB (1947) Germinoma of the pineal; its identity with germinoma (seminoma) of the testis. Cancer Res 7:363–368PubMedGoogle Scholar
  5. Fujimaki T (2009) Central nervous system germ cell tumors: classification, clinical features, and treatment with a historical overview. J Child Neurol 24:1439–1445PubMedCrossRefGoogle Scholar
  6. Fujimaki T, Matsutani M, Funada N, Kirino T, Takakura K, Nakamura O, Tamura A, Sano K (1994) CT and MRI features of intracranial germ cell tumors. J Neurooncol 19:217–226PubMedCrossRefGoogle Scholar
  7. Jennings MT, Gelman R, Hochberg F (1985) Intracranial germ-cell tumors: natural history and pathogenesis. J Neurosurg 63:155–167PubMedCrossRefGoogle Scholar
  8. Katakami H, Hashida S, Yamaguchi H, Yazawa S, Nakano S, Wakisaka S (2003) Central nervous system germ cell tumor, diagnosis and clinical course: development of ultrasensitive HCG-beta measurement and clinical application. Nippon Naibunpi Gakkai Zasshi 79(Suppl):58–60Google Scholar
  9. Kellie SJ, Boyce H, Dunkel IJ, Diez B, Rosenblum M, Brualdi L, Finlay JL (2004) Intensive cisplatin and cyclophosphamide-based chemotherapy without radiotherapy for intracranial germinomas: failure of a primary chemotherapy approach. Pediatr Blood Cancer 43:126–133PubMedCrossRefGoogle Scholar
  10. Khatua S, Dhall G, O’Neil S, Jubran R, Villablance JG, Marachelian A, Nastia A, Lavey R, Olch AJ, Gonzalez I, Gilles F, Nelson M, Panigrahy A, McComb G, Krieger M, Fan J, Sposto R, Finlay J (2010) Treatment of primary CNS germinomatous germ cell tumors with chemotherapy prior to reduced dose whole ventricular and local boost irradiation. Pediatr Blood Cancer 55:42–46PubMedGoogle Scholar
  11. Kochi M, Itoyama Y, Shiraishi S, Kitamura I, Marubayashi T, Ushio Y (2003) Successful treatment of intracranial nongerminomatous malignant germ cell tumors by administering neoadjuvant chemotherapy and radiotherapy before excision of residual tumors. J Neurosurg 99:106–114PubMedCrossRefGoogle Scholar
  12. Kretschmar C, Kleinberg L, Greenberg M, Burger P, Holmes E, Wharam M (2007) Pre-radiation chemotherapy with response-based radiation therapy in children with central nervous system germ cell tumors: a report from the Children’s Oncology Group. Pediatr Blood Cancer 48:285–291PubMedCrossRefGoogle Scholar
  13. Luther N, Edgar MA, Dunkel IJ, Souweidane MM (2006) Correlation of endoscopic biopsy with tumor marker status in primary intracranial germ cell tumors. J Neurooncol 79:45–50PubMedCrossRefGoogle Scholar
  14. Maity A, Shu HK, Janss A, Belasco JB, Rorke L, Phillips PC, Sutton LN, Goldwein JW (2004) Craniospinal radiation in the treatment of biopsy-proven intracranial germinomas: twenty-five years’ experience in a single center. Int J Radiat Oncol Biol Phys 58:1165–1170PubMedCrossRefGoogle Scholar
  15. Matsutani M (2001) Combined chemotherapy and radiation therapy for CNS germ cell tumors – the Japanese experience. J Neurooncol 54:311–316PubMedCrossRefGoogle Scholar
  16. Matsutani M (2010) Long-term follow-up of germinomas treated by ventricular irradiation with 24 Gy and concurrently administered carboplatin and etoposide chemotherapy. Neuro-oncology 12(6):ii28Google Scholar
  17. Matsutani M, Sano K, Takakura K, Fujimaki T, Nakamura O, Funata N, Seto T (1997) Primary intracranial germ cell tumors: a clinical analysis of 153 histologically verified cases. J Neurosurg 86:446–455PubMedCrossRefGoogle Scholar
  18. Rosenblum M, Nakazato Y, Matsutani M (2007) CNS germ cell tumors. In: Luis D (ed) WHO classification of tumours of the central nervous system, 4th edn. International Agency for Research on Cancer, Lyon, 197–204Google Scholar
  19. Russel DS (1944) The pinealoma: its relationship to teratoma. J Pathol Bacteriol 56:145–150CrossRefGoogle Scholar
  20. Sano K, Matsutani M, Seto T (1989) So-called intracranial germ cell tumours: personal experiences and a theory of their pathogenesis. Neurol Res 11:118–126PubMedGoogle Scholar
  21. Shikama N, Ogawa K, Tanaka S, Toita T, Nakamura K, Uno T, Ohnishi H, Itami J, Tada T, Saeki N (2005) Lack of benefit of spinal irradiation in the primary treatment of intracranial germinoma: a multi-institutional, retrospective review of 180 patients. Cancer 104:126–134PubMedCrossRefGoogle Scholar
  22. Silvani A, Eoli M, Salmaggi A, Lamperti E, Fariselli L, Milanesi I, Broggi G, Solero CL, Giombini S, Boiardi A (2005) Combined chemotherapy and radiotherapy for intracranial germinomas in adult patients: a single-institution study. J Neurooncol 71:271–276PubMedCrossRefGoogle Scholar
  23. Sonoda Y, Kumabe T, Sugiyama S, Kanamori M, Yamashita Y, Saito S, Ariga H, Takai Y, Tominaga T (2008) Germ cell tumors in the basal ganglia: problems of early diagnosis and treatment. J Neurosurg Pediatr 2:118–124PubMedCrossRefGoogle Scholar
  24. Surawicz TS, McCarthy BJ, Kupelian V, Jukich PJ, Bruner JM, Davis FG (1999) Descriptive epidemiology of primary brain and CNS tumors: results from the Central Brain Tumor Registry of the United States, 1990–1994. Neuro-Oncology 1:14–25PubMedGoogle Scholar
  25. The Committee of the Brain Tumor Registry of Japan (2009) Report of Brain Tumor Registry of Japan. Neurol Med Chir (Tokyo) 46(Suppl):5Google Scholar
  26. Yoshida J, Sugita K, Kobayashi T, Takakura K, Shitara N, Matsutani M, Tanaka R, Nagai H, Yamada H, Yamashita J (1993) Prognosis of intracranial germ cell tumours: effectiveness of chemotherapy with cisplatin and etoposide (CDDP and VP-16). Acta Neurochir (Wien) 120:111–117CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media B.V. 2012

Authors and Affiliations

  1. 1.Department of NeurosurgerySaitama Medical UniversityMoroyamaJapan

Personalised recommendations