Lynch Syndrome and Gynecologic Tumors: Incidence, Prophylaxis, and Management of Patients with Cancer

Author:

Capasso Ilaria12ORCID,Santoro Angela3ORCID,Lucci Cordisco Emanuela4ORCID,Perrone Emanuele1ORCID,Tronconi Francesca5,Catena Ursula1,Zannoni Gian Franco3,Scambia Giovanni1,Fanfani Francesco1,Lorusso Domenica6,Duranti Simona6ORCID

Affiliation:

1. Gynecologic Oncology Unit, Department of Woman and Child Health and Public Health, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, 00168 Rome, Italy

2. Università Cattolica del Sacro Cuore, 00168 Rome, Italy

3. Gynecopathology and Breast Pathology Unit, Department of Woman and Child Health and Public Health, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, 00168 Rome, Italy

4. Medical Genetics Unit, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, 00168 Rome, Italy

5. Medical Oncology, Università Politecnica delle Marche, 60121 Ancona, Italy

6. Scientific Directorate, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, 00168 Rome, Italy

Abstract

This review provides a comprehensive update on recent evidence regarding gynecologic tumors associated with Lynch Syndrome (LS). Endometrial cancer (EC) and ovarian cancer (OC) are the first and second most common gynecologic malignancies in developed countries, respectively, and LS is estimated to be the hereditary cause in 3% of both EC and OC. Despite the increasing evidence on LS-related tumors, few studies have analyzed the outcomes of LS-related EC and OC stratified by mutational variant. This review aims to provide a comprehensive overview of the literature and comparison between updated international guidelines, to help outline a shared pathway for the diagnosis, prevention, and management of LS. Through the widespread adoption of the immunohistochemistry-based Universal Screening, LS diagnosis and identification of mutational variants could be standardized and recognized by international guidelines as a feasible, reproducible, and cost-effective method. Furthermore, the development of a better understanding of LS and its mutational variants will support our ability to better tailor EC and OC management in terms of prophylactic surgery and systemic treatment in the light of the promising results shown by immunotherapy.

Publisher

MDPI AG

Subject

Cancer Research,Oncology

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