Small Cell Carcinoma of the Ovary, Hypercalcemic Type (SCCOHT): Patient Characteristics, Treatment, and Outcome-A Systematic Review.

Autor: Wens FSPL; Princess Máxima Center for Pediatric Oncology, 3584 CS Utrecht, The Netherlands., Hulsker CCC; Princess Máxima Center for Pediatric Oncology, 3584 CS Utrecht, The Netherlands., Fiocco M; Princess Máxima Center for Pediatric Oncology, 3584 CS Utrecht, The Netherlands.; Mathematical Institute, Leiden University, 2311 EZ Leiden, The Netherlands.; Biomedical Data Science Department, Section Medical Statistics, Leiden University Medical Center, 2333 ZA Leiden, The Netherlands., Zsiros J; Princess Máxima Center for Pediatric Oncology, 3584 CS Utrecht, The Netherlands., Smetsers SE; Princess Máxima Center for Pediatric Oncology, 3584 CS Utrecht, The Netherlands., de Krijger RR; Princess Máxima Center for Pediatric Oncology, 3584 CS Utrecht, The Netherlands.; Department of Pathology, University Medical Center Utrecht, 3584 CG Utrecht, The Netherlands., van der Steeg AFW; Princess Máxima Center for Pediatric Oncology, 3584 CS Utrecht, The Netherlands., Zweemer RP; Department of Gynecologic Oncology, Division of Imaging and Oncology, University Medical Center Utrecht, 3584 CG Utrecht, The Netherlands., Baas IO; Department of Medical Oncology, University Medical Center Utrecht, 3584 CG Utrecht, The Netherlands., Roes EM; Department of Gynecologic Oncology, Erasmus Medical Center Cancer Institute, 3015 GD Rotterdam, The Netherlands., Looijenga LHJ; Princess Máxima Center for Pediatric Oncology, 3584 CS Utrecht, The Netherlands., Gerestein CG; Department of Gynecologic Oncology, Division of Imaging and Oncology, University Medical Center Utrecht, 3584 CG Utrecht, The Netherlands., Mavinkurve-Groothuis AMC; Princess Máxima Center for Pediatric Oncology, 3584 CS Utrecht, The Netherlands.
Jazyk: angličtina
Zdroj: Cancers [Cancers (Basel)] 2023 Jul 26; Vol. 15 (15). Date of Electronic Publication: 2023 Jul 26.
DOI: 10.3390/cancers15153794
Abstrakt: Background: Small-cell carcinoma of the ovary, hypercalcemic type (SCCOHT) is a rare aggressive ovarian malignancy mainly affecting children, adolescents, and young adults. Since the discovery of mutations in the SMARCA4 gene in 2014, SCCOHT has become the subject of extensive investigation. However, international uniform treatment guidelines for SCCOHT are lacking and the outcome remains poor. The aim of this systematic review is to generate an overview of all reported patients with SCCOHT from 1990 onwards, describing the clinical presentation, genetic characteristics, treatment, and outcome.
Methods: A systematic search was performed in the databases Embase, Medline, Web of Science, and Cochrane for studies that focus on SCCOHT. Patient characteristics and treatment data were extracted from the included studies. Survival was estimated using Kaplan-Meier's methodology. To assess the difference between survival, the log-rank test was used. To quantify the effect of the FIGO stage, the Cox proportional hazard regression model was estimated. The chi-squared test was used to study the association between the FIGO stage and the surgical procedures.
Results: Sixty-seven studies describing a total of 306 patients were included. The median patient age was 25 years (range 1-60 years). The patients mostly presented with non-specific symptoms such as abdominal pain and sometimes showed hypercalcemia and elevated CA-125. A great diversity in the diagnostic work-up and therapeutic approaches was reported. The chemotherapy regimens were very diverse, all containing a platinum-based (cisplatin or carboplatin) backbone. Survival was strongly associated with the FIGO stage at diagnosis.
Conclusions: SCCOHT is a rare and aggressive ovarian cancer, with a poor prognosis, and information on adequate treatment for this cancer is lacking. The testing of mutations in SMARCA4 is crucial for an accurate diagnosis and may lead to new treatment options. Harmonization and international collaboration to obtain high-quality data on diagnostic investigations, treatment, and outcome are warranted to be able to develop international treatment guidelines to improve the survival chances of young women with SCCOHT.
Databáze: MEDLINE
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