Treatment Option Overview for Childhood CNS Germ Cell Tumors
- For children older than 3 years and adults, radiation therapy has been an important component of therapy for germinomas and NGGCTs, although the total dose and fields are debated.
- Germinomas are curable with craniospinal irradiation and local site–boost radiation therapy; however, there is a trend in clinical trials to use neoadjuvant or preirradiation chemotherapy to allow reduced doses and volumes of radiation therapy in patients whose tumors have a complete response to chemotherapy to reduce long-term radiation therapy–related effects. Patients with localized germinomas are effectively treated with whole-ventricular irradiation supplemented with tumor site–boost radiation therapy; focal irradiation to the tumor bed, regardless of response to chemotherapy, is considered inadequate treatment.
- For NGGCTs, the combined use of more intensive neoadjuvant chemotherapy followed by craniospinal radiation in clinical trials has resulted in excellent survival rates in the last decade.
- Germ cell tumors (GCTs) arising in the central nervous system, similar to gonadal and extragonadal GCTs, have demonstrated sensitivity to chemotherapy.
|Stage/Tumor Type||Treatment Options|
|Newly diagnosed childhood germinomas||Radiation therapy|
|Neoadjuvant chemotherapy followed by response-based radiation therapy|
|Newly diagnosed childhood teratomas||Surgery|
|Adjuvant therapy, for patients who had a subtotal resection (controversial):|
|—Focal radiation therapy|
|Newly diagnosed childhood nongerminomatous GCTs||Chemotherapy followed by radiation therapy|
|—Surgery, for tumors that do not respond to treatment or for tumors that increase in size|
|—High-dose chemotherapy with stem cell rescue, for high-risk germ cell tumors|
|Recurrent childhood CNS GCTs||Surgery|
|Focal or craniospinal radiation therapy|
|Chemotherapy followed by radiation therapy|
|High-dose chemotherapy with stem cell rescue|
- Osuka S, Tsuboi K, Takano S, et al.: Long-term outcome of patients with intracranial germinoma. J Neurooncol 83 (1): 71-9, 2007. [PUBMED Abstract]
- Allen JC, Kim JH, Packer RJ: Neoadjuvant chemotherapy for newly diagnosed germ-cell tumors of the central nervous system. J Neurosurg 67 (1): 65-70, 1987. [PUBMED Abstract]
- Kellie SJ, Boyce H, Dunkel IJ, et al.: Primary chemotherapy for intracranial nongerminomatous germ cell tumors: results of the second international CNS germ cell study group protocol. J Clin Oncol 22 (5): 846-53, 2004. [PUBMED Abstract]
- Calaminus G, Kortmann R, Worch J, et al.: SIOP CNS GCT 96: final report of outcome of a prospective, multinational nonrandomized trial for children and adults with intracranial germinoma, comparing craniospinal irradiation alone with chemotherapy followed by focal primary site irradiation for patients with localized disease. Neuro Oncol 15 (6): 788-96, 2013. [PUBMED Abstract]
- Calaminus G, Bamberg M, Harms D, et al.: AFP/beta-HCG secreting CNS germ cell tumors: long-term outcome with respect to initial symptoms and primary tumor resection. Results of the cooperative trial MAKEI 89. Neuropediatrics 36 (2): 71-7, 2005. [PUBMED Abstract]
- Joo JH, Park JH, Ra YS, et al.: Treatment outcome of radiation therapy for intracranial germinoma: adaptive radiation field in relation to response to chemotherapy. Anticancer Res 34 (10): 5715-21, 2014. [PUBMED Abstract]