Sex cord stromal tumors in children and adolescents : a first report by the South African Children's Cancer Study Group (1990-2015)

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Authors

Hendricks, Marc
Cois, Annibale
Geel, Jennifer
Van Heerden, Jaques
Naidu, Gita
Du Plessis, Jan
Bassingthwaighte, Mairi
Van Zyl, Anel
Uys, Ronelle
Buchner, Ane

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Lippincott Williams and Wilkins

Abstract

OBJECTIVES : Pediatric sex cord stromal tumors (SCSTs) are extremely rare and there are no reported data from Africa. The authors evaluated the outcomes of children and adolescents with biopsy-proven SCSTs in preparation for the introduction of a national protocol. MATERIALS AND METHODS : Retrospective data were collated from 9 South African pediatric oncology units from January 1990 to December 2015. Kaplan-Meier analysis was performed to estimate overall survival (OS) and event-free survival. RESULTS : Twenty-three patients were diagnosed with SCSTs, 3 male and 20 female individuals, during the study period. Histologies included 1 thecoma, 9 Sertoli-Leydig cell tumors, and 13 juvenile granulosa cell tumors. Stage I tumors predominated (n=14; 60.9%), with 2 stage II (8.7%), 5 stage III (21.7%), and 2 stage IV tumors (8.7%). The upfront resection rate was 91.3% with no reported surgical morbidity or mortality and an OS of 82.1%. Chemotherapy approaches were not standardized. Most children (81.8%), except 2, had recognized platinum-based regimens. Chemotherapy-related toxicity was minimal and acceptable. Assessment of glomerular filtration rate and audiology assessments were infrequent and not standardized. Three patients were lost to follow-up. CONCLUSIONS : Although the numbers in this cohort are small, this study represents the first national cohort in Africa. The 5-year OS of 82.1% was encouraging. Standardized management of rare tumors like SCSTs is critical to improve ensure OS and address potential long-term sequelae.

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Keywords

Sex cord stromal tumor (SCST), Children, Adolescents, Pediatric oncology, South Africa (SA)

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Citation

Hendricks M, Cois A, Geel J, van Heerden J, Naidu G, du Plessis J, Bassingthwaighte M, van Zyl A, Uys R, Bϋchner A, Rowe B, Omar F, Mahlachana N, Thomas K, Vermeulen J, Davidson A, Donald KA, Kruger M. Sex Cord Stromal Tumors in Children and Adolescents: A First Report by The South African Children's Cancer Study Group (1990-2015). Journal of Pediatric Hematology/Oncology 2021 Jul 1;43(5):e619-e624. doi: 10.1097/MPH.0000000000002076.