Abstract
There is little literature to guide therapy in children and young adults with intracranial germ cell tumors. We present 17 consecutively diagnosed intracranial germ cell tumors at The Children's Hospital, Denver, from 1995 to 2001. Of 17 patients, 3 had considerable delay in diagnosis. Two with suprasellar tumors presented with dementia, blindness and pan-hypopituitarism and another with recurrent subarachnoid hemorrhage. Seven had germinoma, three were metastatic at diagnosis. Ten had non-germinomatous germ cell tumors (NGGCT), 5/10 were alpha feto-protein (AFP) positive only, one β-human chorionic growth (βHCG) factor positive only, 3 positive for AFP and βHCG, and 1 malignant teratoma. Therapy for metastatic patients consisted of chemotherapy followed by craniospinal radiation (CSI). Patients with localized disease received chemotherapy followed by focal radiation. Two patients received chemotherapy only, one because she died of sepsis while receiving chemotherapy and one because of neurologic injury incurred during surgery parents elected for no therapy. Three patients have died, one of tumor recurrence, one from a remote complication of surgery and one of sepsis. Twelve patients are alive without evidence of disease from 10 to 68 months (median 31.5 months). All five children with only AFP positivity, treated with chemotherapy and focal radiation are alive without evidence of disease at 10, 16, 22, 41 and 41 months. Thus, there is little evidence that CSI is necessary in non-metastatic germinomas and AFP positive NGGCTs when combined chemotherapy and radiation therapy is used. However, complications of delayed diagnosis, surgery and chemotherapy are important causes of mortality, with only one patient dying of tumor.
Similar content being viewed by others
References
Baranzelli MC, Patte C, Bouffet E, Couanet D, Habrand JL, Portas M, Lejars O, Lutz P, Le Gall E, Kalifa C: Nonmetastatic intracranial germinoma. Cancer 80: 1792–1797, 1997
Shibamoto Y, Sasai K, Oya N, Hiraoka M: Intracranial germinoma: radiation therapy with tumor volume-based dose selection. Radiology 218: 452–456, 2001
Hardenbergh PH, Golden J, Billet A, Scott RM, Shrieve DC, Silver B, Loeffler JS, Tarbell NJ: Intracranial germinoma: the case for lower dose radiation therapy. Int J Radiat Oncol Biol Phys 39: 419–426, 1997
Balmaceda C, Heller G, Rosenblum M, Diez B, Villablanca JG, Kellie S, Maher P, Vlamis V, Walker RW, Leibel S, Finlay J: Chemotherapy without irradiation — a novel approach for newly diagnosed CNS germ cell tumors: results of an international cooperative trial. J Clin Oncol 14: 2908–2915, 1996
Allen JC, CaRosso RC, Donahue B, Nirenberg A: A phase II trial of preirradiation carboplatin in newly diagnosed germinoma of the central nervous system. Cancer 74: 940–944, 1994
Weiner HL, Finlay JL: Surgery in the management of primary intracranial germ cell tumors. Childs Nervous Syst 15: 770–773, 1999
Ushio Y, Kochi M, Kuratsu JI, Itoyama Y, Marubayashi T: Preliminary observations for a new treatment in children with primary intracranial yolk sac tumor or embryonal carcinoma. J Neurosurg 90: 133–137, 1999
Aoyama H, Shirato H, Ikeda J, Fujieda K, Miyasaka K, Sawamura Y: Induction chemotherapy followed by low-dose involved field radiotherapy for intracranial germ cell tumors. J Clin Oncol 20: 857–865, 2002
Robertson PL, DaRosso RC, Allen JC: Improved prognosis of intracranial non-germinoma germ cell tumors with Multimodality therapy. J Neuro-Oncol 32: 71–80, 1997
Baranzelli NC, Patte C, Bouffet E, Portas M, Mechinaud-Lacroix F, Sariban E, Roche H, Kalifa C: An attempt to treat pediatric intracranial AFP and βHCG secreting germ cell tumors with chemotherapy alone. SFOP experience with 18 cases. J Neuro-Oncol 37: 229–239, 1998
Wolden SL, Wara WM, Larson DA, Prados MD, Edwards MS, Sneed PK: Radiation therapy for primary intracranial germ cell tumors. Int J Radiat Oncol Biol Phys 32: 943–949, 1995
Matsutani M, Sano K, Takakura K, Fujimaki T, Nakamura O, Funata N, Seto T: Primary intracranial germ cell tumors: a clinical analysis of 153 histologically verified cases. J Neurosurg 86: 446–455, 1997
Sutton LN, Radcliffe J, Goldwein JW, Phillips P, Janss AJ, Packer RJ, Zhao H: Quality of life of adult survivors of germinomas treated with craniospinal irradiation. Neurosurgery 45: 1292–1297, 1999
Benesch M, Lackner H, Schager H, Gallist S, Frey EM, Urban C: Tumor and treatment related side effects after multimodal therapy of childhood intracranial germ cell tumors. Acta Paediatric 90: 264–270, 2001
Bouffet E, Baranzelli MC, Patte C, Portas M, Edan C, Chastagner P, Mechinaud-Lacroiz F, Kalifa C: Combined treatment modality for intracranial germinomas: results of a multicentre SFOP experience. Br J Cancer 79: 1199–1204, 1999
Sawamura Y, Shirato H, Ikeda J, Tada M, Ishii N, Kato T, Abe H, Fujieda K: Induction chemotherapy followed by reduced-volume radiation therapy for newly diagnosed central nervous system germinoma. J Neurosurg 88: 66–72, 1998
Kuratsu JI, Ushio, Y: Epidemiological study of primary intracranial tumors: a regional survey in kumamoto prefecture in the southern part of Japan. J Neurosurg 84: 946–950, 1996
Matsutani M, Sano K, Takakura K, Fujimaki T, Nakamura O: Combined treatment with chemotherapy and radiation therapy for intracranial germ cell tumors. Childs Nervous Syst 14: 59–62, 1998
Haddock MG, Schild SE, Scheithauser BW, Schomberg PJ: Radiation therapy for histologically confirmed primary central nervous system germinoma. Int J Radiat Oncol Biol Phys 38: 915–923, 1997
Sawamura Y, Ikeda J, Shirato H, Tada M, Abe H: Germ cell tumours of the central nervous system: based on 111 cases and their long-term clinical outcomes. Eur J Cancer 34: 104–110, 1998
Packer RJ, Cohen B, Coney K: Intracranial germ cell tumors. Oncologist 5: 312–320, 2000
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Smith, A., Weng, E., Handler, M. et al. Intracranial Germ Cell Tumors: A Single Institution Experience and Review of the Literature. J Neurooncol 68, 153–159 (2004). https://doi.org/10.1023/B:NEON.0000027670.96412.36
Issue Date:
DOI: https://doi.org/10.1023/B:NEON.0000027670.96412.36