Skip to main content
Log in

Management of primary intracranial germ cell tumors

  • Topic Review
  • Published:
Journal of Neuro-Oncology Aims and scope Submit manuscript

Abstract

Primary intracranial germ cell tumors are rare and usually localized in the pineal and the suprasellar regions. They are divided into the following histologic types: germinoma, teratoma (mature, immature, malignant), choriocarcinoma, embryonal carcinoma, endodermal sinus tumor (yolk sac tumor), and mixed tumors. Clinically, they are manifested with ocular signs or signs of obstructive hydrocephalus. Localized germinomas are treated with radiation therapy and exhibit a relatively good prognosis. Chemotherapy is reserved for disseminated germinomas. Mature teratomas are treated with surgery. The rest of germ cell tumors are managed with various combinations of surgery, chemotherapy, and radiotherapy depending on the tumor type. If the tumors secrete beta-human chorionic gonadotrophin (hCG) or alpha-fetoprotein (FP), these tumor markers can be used to accurately monitor response to treatment. Prognosis is best for germinomas and mature teratomas and worst for choriocarcinomas and embryonal carcinomas.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

  1. Levin VA, Giglio P, Kyritsis AP (2005) The management of gliomas, medulloblastoma, CNS germ cell tumors, and carcinomas metastatic to the CNS. In: Cavalli F, Hansen H, Kaye S (eds) Textbook of medical oncology. Martin Dunitz, London, England, pp 415–430

    Google Scholar 

  2. Jennings MT, Gelman R, Hochberg F (1985) Intracranial germ-cell tumors: natural history and pathogenesis. J Neurosurg 63:155–167

    Article  CAS  PubMed  Google Scholar 

  3. Liang L, Korogi Y, Sugahara T, Ikushima I, Shigematsu Y, Okuda T, Takahashi M, Kochi M, Ushio Y (2002) MRI of intracranial germ-cell tumours. Neuroradiology 44:382–388

    Article  CAS  PubMed  Google Scholar 

  4. Smirniotopoulos JG, Rushing EJ, Mena H (1992) Pineal region masses: differential diagnosis. Radiographics 12:577–596

    CAS  PubMed  Google Scholar 

  5. Kyritsis A (1998) Evaluation and treatment of CNS neoplasms. In: Lechtenberg R, Schutta HS (eds) Neurology practice guidelines. Marcel Dekker, New York, pp 473–496

    Google Scholar 

  6. Okada Y, Nishikawa R, Matsutani M, Louis DN (2002) Hypomethylated X chromosome gain and rare isochromosome 12p in diverse intracranial germ cell tumors. J Neuropathol Exp Neurol 61:531–538

    CAS  PubMed  Google Scholar 

  7. Juric D, Sale S, Hromas RA, Yu R, Wang Y, Duran GE, Tibshirani R, Einhorn LH, Sikic BI (2005) Gene expression profiling differentiates germ cell tumors from other cancers and defines subtype-specific signatures. Proc Natl Acad Sci USA 102:17763–17768

    Article  CAS  PubMed  Google Scholar 

  8. Losi L, Polito P, Hagemeijer A, Buonamici L, Van den BH, Dal CP (1998) Intracranial germ cell tumour (embryonal carcinoma with teratoma) with complex karyotype including isochromosome 12p. Virchows Arch 433:571–574

    Article  CAS  PubMed  Google Scholar 

  9. Asanoma K, Matsuda T, Kondo H, Kato K, Kishino T, Niikawa N, Wake N, Kato H (2003) NECC1, a candidate choriocarcinoma suppressor gene that encodes a homeodomain consensus motif. Genomics 81:15–25

    Article  CAS  PubMed  Google Scholar 

  10. Miyanohara O, Takeshima H, Kaji M, Hirano H, Sawamura Y, Kochi M, Kuratsu J (2002) Diagnostic significance of soluble c-kit in the cerebrospinal fluid of patients with germ cell tumors. J Neurosurg 97:177–183

    Article  CAS  PubMed  Google Scholar 

  11. Nishikawa R, Matsutani M (1998) Immunohistochemical analysis of p53 and p21(WAF1/Cip1) expression in primary intracranial germ cell tumors. Neurosurg Focus 5:e2

    Article  CAS  PubMed  Google Scholar 

  12. Yamagami T, Handa H, Yamashita J, Okumura T, Paine J, Haebara H, Furukawa F (1987) An immunohistochemical study of intracranial germ cell tumours. Acta Neurochir (Wien) 86:33–41

    Article  CAS  Google Scholar 

  13. Hirato J, Nakazato Y (2001) Pathology of pineal region tumors. J Neurooncol 54:239–249

    Article  CAS  PubMed  Google Scholar 

  14. Sano K (1999) Pathogenesis of intracranial germ cell tumors reconsidered. J Neurosurg 90:258–264

    Article  CAS  PubMed  Google Scholar 

  15. Chang T, Teng MM, Guo WY, Sheng WC (1989) CT of pineal tumors and intracranial germ-cell tumors. AJR Am J Roentgenol 153:1269–1274

    CAS  PubMed  Google Scholar 

  16. Konovalov AN, Pitskhelauri DI (2003) Principles of treatment of the pineal region tumors K2003. Surg Neurol 59:250–268

    Article  PubMed  Google Scholar 

  17. 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 19:217–226

    Article  CAS  PubMed  Google Scholar 

  18. Tien RD, Barkovich AJ, Edwards MS (1990) MR imaging of pineal tumors. AJR Am J Roentgenol 155:143–151

    CAS  PubMed  Google Scholar 

  19. Chang IB, Cho BM, Park SH, Yoon DY, Oh SM (2008) Metastatic choriocarcinoma with multiple neoplastic intracranial microaneurysms: case report. J Neurosurg 108:1014–1017

    Article  PubMed  Google Scholar 

  20. Huang CY, Chen CA, Hsieh CY, Cheng WF (2007) Intracerebral hemorrhage as initial presentation of gestational choriocarcinoma: a case report and literature review. Int J Gynecol Cancer 17:1166–1171

    Article  PubMed  Google Scholar 

  21. Vaquero J, Martinez R, Manrique M (2000) Stereotactic biopsy for brain tumors: is it always necessary? Surg Neurol 53:432–437

    Article  CAS  PubMed  Google Scholar 

  22. Shinoda J, Sakai N, Yano H, Hattori T, Ohkuma A, Sakaguchi H (2004) Prognostic factors and therapeutic problems of primary intracranial choriocarcinoma/germ-cell tumors with high levels of HCG. J Neurooncol 66:225–240

    Article  PubMed  Google Scholar 

  23. Blakeley JO, Grossman SA (2006) Management of pineal region tumors. Curr Treat Options Oncol 7:505–516

    Article  PubMed  Google Scholar 

  24. Haas-Kogan DA, Missett BT, Wara WM, Donaldson SS, Lamborn KR, Prados MD, Fisher PG, Huhn SL, Fisch BM, Berger MS, Le QT (2003) Radiation therapy for intracranial germ cell tumors. Int J Radiat Oncol Biol Phys 56:511–518

    Article  PubMed  Google Scholar 

  25. Franzini A, Leocata F, Servello D, Cajola L, Allegranza A, Broggi G (1998) Long-term follow-up of germinoma after stereotactic biopsy and brain radiotherapy: a cell kinetics study. J Neurol 245:593–597

    Article  CAS  PubMed  Google Scholar 

  26. Wolden SL, Wara WM, Larson DA, Prados MD, Edwards MS, Sneed PK (1995) Radiation therapy for primary intracranial germ-cell tumors. Int J Radiat Oncol Biol Phys 32:943–949

    CAS  PubMed  Google Scholar 

  27. Matsutani M (2004) Clinical management of primary central nervous system germ cell tumors. Semin Oncol 31:676–683

    Article  PubMed  Google Scholar 

  28. Echevarria ME, Fangusaro J, Goldman S (2008) Pediatric central nervous system germ cell tumors: a review. Oncologist 13:690–699

    Article  PubMed  Google Scholar 

  29. Bilgin T, Ozan H, Ozuysal S, Ozkan L (2004) Successful salvage therapy of resistant gestational trophoblastic disease with etoposide, methotrexate, actinomycin-D, etoposide, cisplatin (EMA/EP). Arch Gynecol Obstet 269:159–160

    Article  PubMed  Google Scholar 

  30. Chen LP, Cai SM, Fan JX, Li ZT (1995) PEBA regimen (cisplatin, etoposide, bleomycin, and adriamycin) in the treatment of drug-resistant choriocarcinoma. Gynecol Oncol 56:231–234

    Article  CAS  PubMed  Google Scholar 

  31. Rustin GJ, Newlands ES, Begent RH, Dent J, Bagshawe KD (1989) Weekly alternating etoposide, methotrexate, and actinomycin/vincristine and cyclophosphamide chemotherapy for the treatment of CNS metastases of choriocarcinoma. J Clin Oncol 7:900–903

    CAS  PubMed  Google Scholar 

  32. Termrungruanglert W, Kudelka AP, Piamsomboon S, Verschraegen CF, Edwards CL, Lifshitz S, Mante RP, Kavanagh JJ (1996) Remission of refractory gestational trophoblastic disease with high-dose paclitaxel. Anticancer Drugs 7:503–506

    Article  CAS  PubMed  Google Scholar 

  33. Friedman JA, Lynch JJ, Buckner JC, Scheithauer BW, Raffel C (2001) Management of malignant pineal germ cell tumors with residual mature teratoma. Neurosurgery 48:518–522

    Article  CAS  PubMed  Google Scholar 

  34. Kohyama S, Uematsu M, Ishihara S, Shima K, Tamai S, Kusano S (2001) An experience of stereotactic radiation therapy for primary intracranial choriocarcinoma. Tumori 87:162–165

    CAS  PubMed  Google Scholar 

  35. Brandes AA, Pasetto LM, Monfardini S (2000) The treatment of cranial germ cell tumours. Cancer Treat Rev 26:233–242

    Article  CAS  PubMed  Google Scholar 

  36. Akai T, Iizuka H, Kadoya S, Nojima T (1998) Extraneural metastasis of intracranial germinoma with syncytiotrophoblastic giant cells—case report. Neurol Med Chir (Tokyo) 38:574–577

    Article  CAS  Google Scholar 

  37. Sievers EL, Berger M, Geyer JR (1996) Long-term survival of a patient with primary sellar choriocarcinoma with pulmonary metastases: a case report. Med Pediatr Oncol 26:293–295

    Article  CAS  PubMed  Google Scholar 

  38. Kageji T, Nagahiro S, Matsuzaki K, Kanematsu Y, Nakatani M, Okamoto Y, Watanabe T (2007) Successful neoadjuvant synchronous chemo- and radiotherapy for disseminated primary intracranial choriocarcinoma: case report. J Neurooncol 83:199–204

    Article  PubMed  Google Scholar 

  39. 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 99:106–114

    Article  PubMed  Google Scholar 

  40. Sawamura Y, Ikeda J, Shirato H, Tada M, Abe H (1998) Germ cell tumours of the central nervous system: treatment consideration based on 111 cases and their long-term clinical outcomes. Eur J Cancer 34:104–110

    Article  CAS  PubMed  Google Scholar 

  41. Sano K, Matsutani M, Seto T (1989) So-called intracranial germ cell tumours: personal experiences and a theory of their pathogenesis. Neurol Res 11:118–126

    CAS  PubMed  Google Scholar 

  42. 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 86:446–455

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Athanassios P. Kyritsis.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Kyritsis, A.P. Management of primary intracranial germ cell tumors. J Neurooncol 96, 143–149 (2010). https://doi.org/10.1007/s11060-009-9951-z

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11060-009-9951-z

Keywords

Navigation