====== Intracranial germ cell tumor treatment ====== [[Intracranial germ cell tumor]] sometimes presents as bifocal germinoma, and whether bifocal germinoma should be treated as [[synchronous]] or disseminated disease remains unclear. Findings suggest that bifocal germinoma may be considered as a disseminated disease when considering the patterns of failure according to RT fields. In addition, patients who received CSI showed low acute toxicity rates. However, further studies are necessary to confirm these findings. ((Chung SY, Han JW, Kim DS, Yoon HI, Suh CO. Treatment outcomes based on radiation therapy fields for bifocal germinoma: Synchronous or disseminated disease? PLoS One. 2019 Oct 3;14(10):e0223481. doi: 10.1371/journal.pone.0223481. eCollection 2019. PubMed PMID: 31581215. )). ===== Radiotherapy ===== [[Radiotherapy]] is the preferred treatment of choice in [[intracranial germ cell tumor]]s. It has been thoroughly studied to what extent radiation doses and fields can be limited in order to avoid side effects in these young patients. Reduced dose and volume of extended-field rather than total dose or involved-field will be critical to decrease the late toxicities. [[Upfront chemotherapy]] could be beneficial for the patients with complete response to minimize the RT dose down to 30 Gy. [[Prospective]] [[trial]]s focused on de-intensification of the extended-field RT are warranted. ((Lee JH, Eom KY, Phi JH, Park CK, Kim SK, Cho BK, Kim TM, Heo DS, Hong KT, Choi JY, Kang HJ, Shin HY, Choi SH, Lee ST, Park SH, Wang KC, Kim IH. Long-Term Outcomes and Sequelae Analysis of Intracranial Germinoma: Need to Reduce the Extended-Field Radiotherapy Volume and Dose to Minimize Late Sequelae. Cancer Res Treat. 2021 Jan 13. doi: 10.4143/crt.2020.1052. Epub ahead of print. PMID: 33494128.)). ===== Chemotherapy ===== Surgery is limited to biopsy for proof of histology. ---- Timely initiation of [[chemotherapy]] is imperative to rapidly reduce tumor bulk in children with [[intracranial germinoma]] and limits the need for [[ventriculoperitoneal shunt]] insertions. In children in whom CSF diversion is required, [[hydrocephalus]] may be successfully managed with a temporary [[external ventricular drain]] ± [[endoscopic third ventriculostomy]] ((Ronsley R, Bouffet E, Dirks P, Drake J, Kulkarni A, Bartels U. Successful management of symptomatic [[hydrocephalus]] using a temporary [[external ventricular drain]] with or without [[endoscopic third ventriculostomy]] in [[pediatric patient]]s with [[germinoma]]. J Neurosurg. 2021 Dec 3:1-6. doi: 10.3171/2021.8.JNS211443. Epub ahead of print. PMID: 35238528.)). ---- Currently used upfront [[chemotherapy]] followed by reduced-dose, reduced-volume RT appears acceptable, when whole-ventricle RT for pineal or [[suprasellar tumor]]s and, at minimum, whole-brain RT for basal ganglia/thalamus lesions are applied. Martens et al. presented a single institution analysis of patients with intracranial germinoma and analyze the long-term outcome with special regard to the quality of life. Thirty-three patients with intracranial germinomas were analyzed by chart review, telephone interview, and neurological assessment. Additionally, a survey on quality of life was performed. The 10-year overall survival rate was 82.1 % at a mean follow-up of 141 (22-306) months. Three quarters (76 %) of the patients reached a favorable neurological outcome on the Modified Rankin Scale (mRS 0-2). However, the self-reported quality of life was significantly worse in germinoma patients compared with a healthy control group (p < 0.001). Surgical resection of the tumor led to no improvement regarding overall survival, neurological outcome, and quality of life. In terms of cognitive functioning, patients with tumor resection were significantly more impaired than biopsied patients (p = 0.04). Although germinomas are efficiently treatable tumors, the restrictions in quality of life in these often young patients are considerable, including financial difficulties. There seems no justification for tumor resection in newly diagnosed cases suspicious for germinoma as the cognitive outcome is worse than in biopsied patients, and there is no effect on overall survival ((Martens T, Rotermund R, Zu Eulenburg C, Westphal M, Flitsch J. Long-term follow-up and quality of life in patients with intracranial germinoma. Neurosurg Rev. 2014 Jul;37(3):445-50; discussion 451. doi: 10.1007/s10143-014-0544-8. Epub 2014 Apr 9. PubMed PMID: 24715277.)). ===== References =====