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Updated: Aug 19, 2025

Murine Model for Non-invasive Imaging to Detect and Monitor Ovarian Cancer Recurrence
Published on: November 2, 2014
Update on new treatments for rare ovarian tumours
Stanislas Quesada1,2, Marta Bini1,3, Coriolan Lebreton4
1Centre Léon Bérard, Lyon, France.
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.
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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