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Review
. 2023 Feb 1;35(1):27-33.
doi: 10.1097/GCO.0000000000000836. Epub 2022 Nov 18.

Update on new treatments for rare ovarian tumours

Stanislas Quesada  1   2 , Marta Bini  1   3 , Coriolan Lebreton  4 , Isabelle Ray-Coquard  1
Affiliations
Review

Update on new treatments for rare ovarian tumours

Stanislas Quesada et al. Curr Opin Obstet Gynecol. .

Abstract

Purpose of review: In spite of their rarity when considered individually, the sum of all rare ovarian tumours (ROT) represent almost half of all ovarian malignancies. As such, their appropriate inclusion within dedicated clinical trials is essential for enhanced management.

Recent findings: Supported by institutional expert national (e.g. TMRG) and international (e.g. ESGO) networks and owing to national (e.g. ARCAGY-GINECO) and international (e.g. ENGOT) collaborations dedicated to clinical research, the last few years have shown increased number of clinical trials dedicated to ROT. These either were based on specific molecular features of ROT (e.g. expression of oestrogen receptors for low-grade serous ovarian carcinomas and anastrazole evaluation in the PARAGON trial) or on the evaluation of innovative therapies (e.g. pembrolizumab within the ROT cohort from the AcSé Pembrolizumab multicentric basket trial). Furthermore, recent years have also shown the advent of randomized clinical trials. For instance, the ALIENOR trial positioned weekly paclitaxel as a new option for relapsed sex cord-stromal tumours, while the GOG281/LOGS trial raised trametinib as a new standard-of-care option for recurrent low-grade serous carcinomas.

Summary: The last few years have exhibited a paradigm shift towards the possibility to develop dedicated trials for ROT, owing to international collaborations supported by institutional networks. Current trials, molecular-driven and based on innovative designs, are highly promising, as they may bring ROT management towards more personalized medicine.

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Conflict of interest statement

S.Q., M.B. and C.L. declare no conflicts of interest. I.R.C. declares the following disclosures: Abbvie, Agenus, Advaxis, BMS, PharmaMar, Genmab, Pfizer, AstraZeneca, Roche, GSK, MSD, Deciphera, Mersena, Merck Sereno, Novartis, Amgen, Tesaro and Clovis; honoraria (institution) from GSK, MSD, Roche and BMS; advisory/consulting fees from Abbvie, Agenus, Advaxis, BMS, PharmaMar, Genmab, Pfizer, AstraZeneca, Roche/Genentech, GSK, MSD, Deciphera, Mersena, Merck Sereno, Novartis, Amgen, Tesaro and Clovis; research grant/funding (self) from MSD, Roche and BMS; research grant/funding (institution) from MSD, Roche, BMS, Novartis, Astra Zeneca and Merck Sereno; and travel support from Roche and AstraZeneca and GSK.

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References

    1. Ray-Coquard I, Trama A, Seckl MJ, et al. . Rare ovarian tumours: epidemiology, treatment challenges in and outside a network setting. Eur J Surg Oncol 2019; 45:67–74. - PubMed
    1. Debuquoy C, Romeo C, Vanacker H, et al. . Rare ovarian tumors: an update on diagnosis and treatment. Int J Gynecol Cancer 2020; 30:879–887. - PubMed
    1. Colombo N, Sessa C, du Bois A, et al. . ESMO-ESGO consensus conference recommendations on ovarian cancer: pathology and molecular biology, early and advanced stages, borderline tumours and recurrent disease. Ann Oncol 2019; 30:672–705. - PubMed
    1. Bookman MA, Okamoto A, Stuart G, et al. . Harmonising clinical trials within the Gynecologic Cancer InterGroup: consensus and unmet needs from the Fifth Ovarian Cancer Consensus Conference. Ann Oncol 2017; 28:viii30–viii35. - PMC - PubMed
    1. Gershenson DM, Okamoto A, Ray-Coquard I. Management of rare ovarian cancer histologies. J Clin Oncol 2019; 37:2406–2415. - PubMed