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Childhood Central Nervous System Germ Cell Tumors Treatment (PDQ®)

Treatment Option Overview for Childhood CNS Germ Cell Tumors

Teratomas, germinomas, and other nongerminomatous germ cell tumors (NGGCTs) have differing prognoses and require different treatment regimens. Studies have observed the following:[1-5]

  • 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 germinoma should receive whole-ventricular irradiation, rather than focal irradiation to the tumor bed, regardless of response to chemotherapy.
  • 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.
Table 4. Treatment Options for Childhood Central Nervous System (CNS) Germ Cell Tumors (GCTs)
Stage/Tumor TypeTreatment Options
Newly diagnosed childhood germinomasRadiation therapy
Neoadjuvant chemotherapy followed by response-based radiation therapy
Newly diagnosed childhood teratomasSurgery
Adjuvant therapy, for patients who had a subtotal resection (controversial):
—Focal radiation therapy
—Chemotherapy
—Stereotactic radiosurgery
Newly diagnosed childhood nongerminomatous GCTsChemotherapy and radiation therapy
Adjuvant 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 GCTsSurgery
Focal or craniospinal radiation therapy
Chemotherapy followed by radiation therapy
High-dose chemotherapy with stem cell rescue

References

  1. 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]
  2. 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]
  3. 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]
  4. 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]
  5. 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]
  • Updated: December 22, 2014