Risk-Reducing Gynecological Surgery in Lynch Syndrome: Results of an International Survey from the Prospective Lynch Syndrome Database

Mev Dominguez-Valentin 1, Toni T Seppälä 2 3, Christoph Engel 4, Stefan Aretz 5 6, Finlay Macrae 7 8, Ingrid Winship 7 8, Gabriel Capella 9 10, Huw Thomas 11, Eivind Hovig 1 12, Maartje Nielsen 13, Rolf H Sijmons 14, Lucio Bertario 15, Bernardo Bonanni 16, Maria Grazia Tibiletti 17, Giulia Martina Cavestro 18, Miriam Mints 19, Nathan Gluck 20, Lior Katz 21, Karl Heinimann 22, Carlos A Vaccaro 23, Kate Green 24, Fiona Lalloo 24, James Hill 25, Wolff Schmiegel 26, Deepak Vangala 27, Claudia Perne 5 6, Hans-Georg Strauß 28, Johanna Tecklenburg 29, Elke Holinski-Feder 30 31, Verena Steinke-Lange 30 31, Jukka-Pekka Mecklin 32 33, John-Paul Plazzer 7, Marta Pineda 9 10, Matilde Navarro 9 10, Joan Brunet Vidal 9 10, Revital Kariv 20, Guy Rosner 20, Tamara Alejandra Piñero 23, María Laura Gonzalez 23, Pablo Kalfayan 23, Julian R Sampson 34, Neil A J Ryan 24, D Gareth Evans 24, Pål Møller 1, Emma J Crosbie 24

Affiliations

PMID: 32708519
DOI: 10.3390/jcm9072290

Free article

Abstract

Purpose: To survey risk-reducing hysterectomy and bilateral salpingo-oophorectomy (BSO) practice and advice regarding hormone replacement therapy (HRT) in women with Lynch syndrome.

Methods: We conducted a survey in 31 contributing centers from the Prospective Lynch Syndrome Database (PLSD), which incorporates 18 countries worldwide. The survey covered local policies for risk-reducing hysterectomy and BSO in Lynch syndrome, the timing when these measures are offered, the involvement of stakeholders and advice regarding HRT.

Results: Risk-reducing hysterectomy and BSO are offered to path_MLH1 and path_MSH2 carriers in 20/21 (95%) contributing centers, to path_MSH6 carriers in 19/21 (91%) and to path_PMS2 carriers in 14/21 (67%). Regarding the involvement of stakeholders, there is global agreement (~90%) that risk-reducing surgery should be offered to women, and that this discussion may involve gynecologists, genetic counselors and/or medical geneticists. Prescription of estrogen-only HRT is offered by 15/21 (71%) centers to women of variable age range (35-55 years).

Conclusions: Most centers offer risk-reducing gynecological surgery to carriers of path_MLH1, path_MSH2 and path_MSH6 variants but less so for path_PMS2 carriers. There is wide variation in how, when and to whom this is offered. The Manchester International Consensus Group developed recommendations to harmonize clinical practice across centers, but there is a clear need for more research.

Keywords: Lynch syndrome; endometrial cancer; ovarian cancer; risk-reducing surgery.