TY - JOUR A1 - Calaminus, Gabriele A1 - Schneider, Dominik T. A1 - Schweinitz, Dietrich von A1 - Jürgens, Heribert A1 - Infed, Nacera A1 - Schönberger, Stefan A1 - Olson, Thomas A. A1 - Albers, Peter A1 - Vokuhl, Christian A1 - Stein, Raimund A1 - Looijenga, Leendert A1 - Sehouli, Jalid A1 - Metzelder, Martin A1 - Claviez, Alexander A1 - Dworzak, Michael A1 - Eggert, Angelika A1 - Fröhlich, Birgit A1 - Gerber, Nicolas U. A1 - Kratz, Christian P. A1 - Faber, Jörg A1 - Klingebiel, Thomas A1 - Harms, Dieter A1 - Göbel, Ulrich T1 - Age-dependent presentation and clinical course of 1465 patients aged 0 to less than 18 years with ovarian or testicular germ cell tumors; data of the MAKEI 96 protocol revisited in the light of prenatal germ cell biology T2 - Cancers N2 - Objective: To evaluate prognostic factors in pediatric patients with gonadal germ cell tumors (GCT). Methods: Patients <18 years with ovarian and testicular GCT (respectively OGCT and TGCT) were prospectively registered according to the guidelines of MAKEI 96. After resection of the primary tumor, patients staged ≥II received risk-stratified cisplatin-based combination chemotherapy. Patients were analyzed in respect to age (six age groups divided into 3-year intervals), histology, stage, and therapy. The primary end point was overall survival. Results: Between January 1996 and March 2016, the following patients were registered: 1047 OGCT, of those, 630 had ovarian teratoma (OTER) and 417 had malignant OGCT (MOGCT); and 418 TGCT, of those, 106 had testicular teratoma (TTER) and 312 had malignant TGCT (MTGCT). Only in MTGCT, older age correlated with a higher proportion of advanced tumors. All 736 teratomas and 240/415 stage I malignant gonadal GCT underwent surgery and close observation alone. In case of watchful waiting, the progression rate of OGCT was higher than that of TGCT. However, death from disease was reported in 8/417 (1.9%) MOGCT and 8/312 (2.6%) MTGCT irrespective of adjuvant chemotherapy and repeated surgery. Conclusions: The different pathogenesis and histogenesis of gonadal GCT reflects sex- and age-specific patterns that define clinically relevant risk groups. Therefore, gender and age should be considered in further research on the biology and clinical practice of pediatric gonadal GCT. KW - germ cell tumors KW - ovary KW - testis KW - children and adolescents KW - age KW - sex KW - histology Y1 - 2020 UR - http://publikationen.ub.uni-frankfurt.de/frontdoor/index/index/docId/53422 UR - https://nbn-resolving.org/urn:nbn:de:hebis:30:3-534225 SN - 2072-6694 N1 - This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited VL - 12 IS - 611 PB - MDPI CY - Basel ER -