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Review

Rare tumors in gynaecological cancers and the lack of therapeutic options and clinical trials

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Pages 71-83 | Received 09 May 2016, Accepted 21 Nov 2016, Published online: 05 Dec 2016
 

ABSTRACT

Introduction: Up to 50% of gynecological cancers can be considered rare according to the Surveillance of Rare Cancers in Europe (RARECARE) consortium definition of an incidence of less than 6 cases per 100 000 people. These cancers usually have a poor prognosis as they are often delayed in their diagnosis and treatment due to the lack of knowledge.

Areas covered: This review briefly addresses the current state of management and the lack of effective treatment strategies for the most commonly seen rare gynecological malignancies. It also highlights the challenges surrounding attempts to harmonize treatment practices and the role of the international medical community.

Expert opinion: Given their rarity, biological and clinical data are lacking for many gynecological cancers. Current efforts are on-going to improve care of these patients, including the development of international consortia, prospective databases with biobanking, acceptance of novel clinical trial design and education of the medical field as well as improvement of patient awareness.

Article highlights

  • Almost fifty percent of gynaecological cancers meet the definition of rare cancers.

  • A multidisciplinary approach is important for the diagnosis and treatment of these rare cancers.

  • Research and drug development for these cancers is lacking due to their rare nature.

  • International consortia are important for the study of these rare cancers.

  • More resources dedicated to the education of the public, primary care physicians and general oncologists are required to ensure appropriate diagnosis and treatment of these patients.

This box summarizes key points contained in the article.

Acknowledgments

We would like to thank the GCIG and the Rare Tumour Working Group for its continued commitment to advancing care in rare gynaecological cancers.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Additional information

Funding

This paper was not funded.

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