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

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 
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.
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Author:Gabriele Calaminus, Dominik T. Schneider, Dietrich von Schweinitz, Heribert Jürgens, Nacera Infed, Stefan Schönberger, Thomas A. Olson, Peter Albers, Christian Vokuhl, Raimund Stein, Leendert Looijenga, Jalid Sehouli, Martin Metzelder, Alexander Claviez, Michael Dworzak, Angelika Eggert, Birgit Fröhlich, Nicolas U. Gerber, Christian P. Kratz, Jörg Faber, Thomas Klingebiel, Dieter Harms, Ulrich Göbel
URN:urn:nbn:de:hebis:30:3-534225
DOI:http://dx.doi.org/10.3390/cancers12030611
ISSN:2072-6694
Parent Title (English):Cancers
Publisher:MDPI
Place of publication:Basel
Document Type:Article
Language:English
Date of Publication (online):2020/03/06
Date of first Publication:2020/03/06
Publishing Institution:Universitätsbibliothek Johann Christian Senckenberg
Release Date:2020/04/30
Tag:age; children and adolescents; germ cell tumors; histology; ovary; sex; testis
Volume:12
Issue:611
Note:
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
HeBIS PPN:465063918
Institutes:Medizin
Dewey Decimal Classification:610 Medizin und Gesundheit
Sammlungen:Universitätspublikationen
Licence (German):License LogoCreative Commons - Namensnennung 4.0

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