Update on new treatments for rare ovarian tumours

Stanislas Quesada1,2, Marta Bini1,3, Coriolan Lebreton4

  • 1Centre Léon Bérard, Lyon, France.

Abstract

Insights

Rare ovarian tumors (ROT) are nearly half of all ovarian cancers. Recent clinical trials, focusing on molecular features and innovative therapies, are improving management for these rare malignancies.

Area of Science:

  • Gynecologic Oncology
  • Rare Tumors
  • Clinical Trial Design

Background:

  • Rare ovarian tumors (ROT) constitute a significant proportion of all ovarian malignancies.
  • Effective management of ROT necessitates their inclusion in dedicated clinical trials.

Approach:

  • Recent years have seen an increase in clinical trials for ROT, supported by national and international research networks.
  • Trials are increasingly molecular-driven, targeting specific tumor features (e.g., estrogen receptor expression) or evaluating novel therapies (e.g., targeted agents, immunotherapy).
  • The advent of randomized clinical trials signifies progress in establishing new treatment standards for specific ROT subtypes.

Key Points:

  • The PARAGON trial investigated anastrozole for low-grade serous ovarian carcinomas based on estrogen receptor expression.
  • The AcSé Pembrolizumab trial evaluated pembrolizumab in a rare tumor cohort, including ROT.
  • The ALIENOR trial established weekly paclitaxel as an option for relapsed sex cord-stromal tumors.
  • The GOG281/LOGS trial identified trametinib as a standard of care for recurrent low-grade serous carcinomas.

Conclusions:

  • International collaborations and institutional networks have enabled the development of dedicated trials for ROT.
  • Current molecular-driven trials with innovative designs hold promise for advancing personalized medicine in ROT management.

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