Abstract：Objective To investigate the clinical features of pediatric patients with intracranial primary non‐germinomatous germ cell tumors (NGGCT) and evaluate the treatment outcomes and prognostic factors of NGGCT. Methods Clinical data of 40 children with NGGCT who were treated with radiotherapy (RT) at our department between November 2008 and June 2019 were retrospectively analyzed. Ninety percent of them received craniospinal irradiation (CSI). All children received platinum‐based chemotherapy. Survival analysis was conducted using the Kaplan‐Meier estimate. The prognostic factors were analyzed by log‐rank test. Results The primary sites were pineal gland, sellar / suprasellar region and basal ganglia. The median age of onset was 108 months (20‐204 months). The median follow‐up time was 33 months (8‐131 months), and the 3‐year and 5‐year overall survival (OS) rates were 82.0%. The 3‐year and 5‐year progression‐free survival (PFS) rates were 78.6% and 73.0%. Univariate analysis showed that increased alpha‐fetoprotein (AFP) (P=0.02), age at first diagnosis>10 years (P=0.006), metastasis at first diagnosis (P<0.001), and the pathological type (choriocarcinoma, yolk sac tumor and / or embryonal carcinoma) (P=0.036) were independent adverse prognostic factors. Conclusions Increased AFP, age>10 years at first diagnosis, tumor metastasis and pathological type were independent adverse prognostic factors of NGGCT. The overall prognosis of NGGCT children is worse than that of their counterparts with germinoma, and multidisciplinary intensive therapy is needed to improve survival.
Shi Xuejiao,Zhou Renhua,Lu Dongqing et al. Analysis of therapeutic effect of pediatric patients with intracranial primary non‐germinomatous germ cell tumors[J]. Chinese Journal of Radiation Oncology, 2022, 31(10): 879-883.
[1] Echevarría ME, Fangusaro J, Goldman S.Pediatric central nervous system germ cell tumors: a review[J]. Oncologist, 2008,13(6):690-699. DOI: 10.1634/theoncologist.2008-0037. [2] Borg M.Germ cell tumours of the central nervous system in children-controversies in radiotherapy[J]. Med Pediatr Oncol, 2003,40(6):367-374. DOI: 10.1002/mpo.10285. [3] Pruitt R, DaSilva NS, Cappellano A, et al. Relapse and outcome patterns of patients with central nervous system mixed malignant germ cell tumors treated without irradiation: findings from the third international central nervous system (CNS) germ cell tumor (GCT) study[J]. Pediatr Blood Cancer, 2015,62(11):1920-1924. DOI: 10.1002/pbc.25606. [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[J]. Neuro Oncol, 2013,15(6):788-796. DOI: 10.1093/neuonc/not019. [5] Calaminus G, Frappaz D, Kortmann RD, et al.Outcome of patients with intracranial non-germinomatous germ cell tumors-lessons from the SIOP-CNS-GCT-96 trial[J]. Neuro Oncol, 2017,19(12):1661-1672. DOI: 10.1093/neuonc/nox122. [6] Shimizu H, Motomura K, Ohka F, et al.Long-term survival in patients with primary intracranial germ cell tumors treated with surgery, platinum-based chemotherapy, and radiotherapy: a single-institution study[J]. J Neurosurg, 2020:1-9. DOI: 10.3171/2020.6.JNS20638. [7] Lian X, Hou X, Yan J, et al.Treatment outcomes of intracranial germinoma: a retrospective analysis of 170 patients from a single institution[J]. J Cancer Res Clin Oncol, 2019,145(3):709-715. DOI: 10.1007/s00432-018-2743-0. [8] Murray MJ, Bartels U, Nishikawa R, et al.Consensus on the management of intracranial germ-cell tumours[J]. Lancet Oncol, 2015,16(9):e470-e477. DOI: 10.1016/S1470-2045(15)00244-2. [9] Goldman S, Bouffet E, Fisher PG, et al.Phase II trial assessing the ability of neoadjuvant chemotherapy with or without second-look surgery to eliminate measurable disease for nongerminomatous germ cell tumors: a children's oncology group study[J]. J Clin Oncol, 2015,33(22):2464-2471. DOI: 10.1200/JCO.2014.59.5132. [10] Li B, Feng J, Chen L, et al.Relapse pattern and quality of life in patients with localized basal ganglia germinoma receiving focal radiotherapy, whole-brain radiotherapy, or craniospinal irradiation[J]. Radiother Oncol, 2021,158:90-96. DOI: 10.1016/j.radonc.2021.02.009. [11] Fangusaro J, Wu S, MacDonald S, et al. Phase II trial of response-based radiation therapy for patients with localized CNS nongerminomatous germ cell tumors: a children's oncology group study[J]. J Clin Oncol, 2019,37(34):3283-3290. DOI: 10.1200/JCO.19.00701. [12] Matsutani M, Sano K, Takakura K, et al.Primary intracranial germ cell tumors: a clinical analysis of 153 histologically verified cases[J]. J Neurosurg, 1997,86(3):446-455. DOI: 10.3171/jns.1997.86.3.0446. [13] Frappaz D, Dhall G, Murray MJ, et al.EANO, SNO and Euracan consensus review on the current management and future development of intracranial germ cell tumors in adolescents and young adults[J]. Neuro Oncol, 2022,24(4):516-527. DOI: 10.1093/neuonc/noab252. [14] Nakamura H, Takami H, Yanagisawa T, et al.The Japan society for neuro-oncology guideline on the diagnosis and treatment of central nervous system germ cell tumors[J]. Neuro Oncol, 2022,24(4):503-515. DOI: 10.1093/neuonc/noab242. [15] Mann JR, Gray ES, Thornton C, et al.Mature and immature extracranial teratomas in children: the UK children's cancer study group experience[J]. J Clin Oncol, 2008,26(21):3590-3597. DOI: 10.1200/JCO.2008.16.0622. [16] Plant AS, Chi SN, Frazier L.Pediatric malignant germ cell tumors: a comparison of the neuro-oncology and solid tumor experience[J]. Pediatr Blood Cancer, 2016,63(12):2086-2095. DOI: 10.1002/pbc.26165. [17] Fujimaki T.Central nervous system germ cell tumors: classification, clinical features, and treatment with a historical overview[J]. J Child Neurol, 2009,24(11):1439-1445. DOI: 10.1177/0883073809342127. [18] Breen WG, Blanchard MJ, Rao AN, et al. Optimal radiotherapy target volumes in intracranial nongerminomatous germ cell tumors: Long-term institutional experience with chemotherapy, surgery,dose- and field-adapted radiotherapy[J]. Pediatr Blood Cancer, 2017,64(11)DOI: 10.1002/pbc.26637.
2022, Vol. 31 
