Abstract

Intracranial germ cell tumor (iGCT) represents a rare and heterogeneous group, with variable incidence and diverse treatment strategies. Although multiagent chemotherapy with reduced radiotherapy strategy has been applied by several cooperative groups in North America and Western Europe, there is a paucity of data to understand if this combined regimen is suitable in low-middle income countries (LMIC). We evaluate the outcome in a cohort of iGCT treated by SIOP-CNS-GCT-96 strategy at hospital J.P Garrahan in Argentina over the last 20years. Radiation field and dose included focal radiotherapy (FRT) before 2009 or focal radiotherapy plus whole ventricular radiotherapy (WVRT) after 2009 for localized germinoma and FRT or FRT plus WVRT or CSI for non germinomatous germ cell tumors (NGGCT) RESULTS: Sixty iGCT were identified; 39 germinoma and 21 NGGCT. Median follow-up was 6.57years (range 0.13-20.5). 5-year PFS and OS were 83.5% (95% CI [165.53-223.2]) and 88.7% (95% CI [169.84-223.2]) for the germinoma group, while for the NGGCT group were 75% (95% CI [133.27-219.96]) and 64.2% (95% CI [107.38-201.81]) respectively. The localized germinoma group showed poor results between 2000 and 2009 with 5-year PFS and OS of 69 and 75% respectively, and an excellent outcome between 2010 and 2019 with a 5-years PFS and OS of 92.8 and 100%. A univariable analysis identified this difference in survival as related to the field of radiotherapy, specifically whole ventricular radiotherapy. FRT increased the risk of recurrence in localized germinoma, involving not only ventricular relapses; but spinal cord and disseminated disease as well. There were no relapses of localized NGGCT after FRT and FRT plus WVRT. Herein we demonstrate that intensive chemotherapy followed by FRT plus WVRT for germinoma is a feasible and effective strategy, warranting further study in the developing world.

Highlights

  • Intracranial germ cell tumor represents a rare and heterogeneous group, with variable incidence and diverse treatment strategies across the continents [1,2]

  • Neoadjuvant chemotherapy was based on Société Internationale d'Oncologie Pédiatrique (SIOP)-CNS-GCT 96 (2 courses of carboplatin/etoposide alternating with Ifosfamide/Etoposidefor germinoma and 4 courses of Cisplatin/Etoposide/Ifosfamide for non germinomatous germ cell tumors (NGGCT)) in 55 patients while 4 patients received 4 courses of Carboplatin/Etoposide for better diabetes insipidusmanagement

  • Discussion we report the outcome of localized germinoma and NGGCT treated with multi-agent chemotherapy, 11 followed by reduced-volume radiation during 20 years in one center in Argentina

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Summary

Introduction

Intracranial germ cell tumor (iGCT) represents a rare and heterogeneous group, with variable incidence and diverse treatment strategies across the continents [1,2]. Alpha-fetoprotein (AFP) and human chorionic gonadotropin-beta (hCGβ) classified iGCT in Europe and the Americas, in two groups: germinoma and non-germinomatous germ-cell tumours (NGGCT). This classification system reflects the excellent overall survival for the first group, and the inferior survival for the second one. Intracranial germ cell tumor (iGCT) represents a rare and heterogeneous group, with variable incidence and diverse treatment strategies.

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