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

Mev Dominguez-Valentin; Toni T Seppälä; Christoph Engel; Stefan Aretz; Finlay Macrae; Ingrid Winship; Gabriel Capella; Huw Thomas; Eivind Hovig; Maartje Nielsen; Rolf H Sijmons; Lucio Bertario; Bernardo Bonanni; Maria Grazia Tibiletti; Giulia Martina Cavestro; Miriam Mints; Nathan Gluck; Lior Katz; Karl Heinimann; Carlos A Vaccaro; Kate Green; Fiona Lalloo; James Hill; Wolff Schmiegel; Deepak Vangala; Claudia Perne; Hans-Georg Strauß; Johanna Tecklenburg; Elke Holinski-Feder; Verena Steinke-Lange; Jukka-Pekka Mecklin; John-Paul Plazzer; Marta Pineda; Matilde Navarro; Joan Brunet Vidal; Revital Kariv; Guy Rosner; Tamara Alejandra Piñero; María Laura Gonzalez; Pablo Kalfayan; Julian R Sampson; Neil A J Ryan; D Gareth Evans; Pål Møller; Emma J Crosbie

doi:10.3390/jcm9072290

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

Mev Dominguez-Valentin, Toni T Seppälä, Christoph Engel, Stefan Aretz, Finlay Macrae, Ingrid Winship, Gabriel Capella, Huw Thomas, Eivind Hovig, Maartje Nielsen, Rolf H Sijmons, Lucio Bertario, Bernardo Bonanni, Maria Grazia Tibiletti, Giulia Martina Cavestro, Miriam Mints, Nathan Gluck, Lior Katz, Karl Heinimann, Carlos A VaccaroKate Green, Fiona Lalloo, James Hill, Wolff Schmiegel, Deepak Vangala, Claudia Perne, Hans-Georg Strauß, Johanna Tecklenburg, Elke Holinski-Feder, Verena Steinke-Lange, Jukka-Pekka Mecklin, John-Paul Plazzer, Marta Pineda, Matilde Navarro, Joan Brunet Vidal, Revital Kariv, Guy Rosner, Tamara Alejandra Piñero, María Laura Gonzalez, Pablo Kalfayan, Julian R Sampson, Neil A J Ryan, D Gareth Evans, Pål Møller, Emma J Crosbie

Bristol Medical School (THS)

Research output: Contribution to journal › Article (Academic Journal) › peer-review

17 Citations (Scopus)

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.

Original language	English
Journal	Journal of Clinical Medicine
Volume	9
Issue number	7
DOIs	https://doi.org/10.3390/jcm9072290
Publication status	Published - 18 Jul 2020

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Dominguez-Valentin, M., Seppälä, T. T., Engel, C., Aretz, S., Macrae, F., Winship, I., Capella, G., Thomas, H., Hovig, E., Nielsen, M., Sijmons, R. H., Bertario, L., Bonanni, B., Tibiletti, M. G., Cavestro, G. M., Mints, M., Gluck, N., Katz, L., Heinimann, K., ... Crosbie, E. J. (2020). Risk-Reducing Gynecological Surgery in Lynch Syndrome: Results of an International Survey from the Prospective Lynch Syndrome Database. Journal of Clinical Medicine, 9(7). https://doi.org/10.3390/jcm9072290

@article{569bf33a650144b0847c1e334ae773c1,

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

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 (\textasciitilde{}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.",

author = "Mev Dominguez-Valentin and Sepp{\"a}l{\"a}, \{Toni T\} and Christoph Engel and Stefan Aretz and Finlay Macrae and Ingrid Winship and Gabriel Capella and Huw Thomas and Eivind Hovig and Maartje Nielsen and Sijmons, \{Rolf H\} and Lucio Bertario and Bernardo Bonanni and Tibiletti, \{Maria Grazia\} and Cavestro, \{Giulia Martina\} and Miriam Mints and Nathan Gluck and Lior Katz and Karl Heinimann and Vaccaro, \{Carlos A\} and Kate Green and Fiona Lalloo and James Hill and Wolff Schmiegel and Deepak Vangala and Claudia Perne and Hans-Georg Strau{\ss} and Johanna Tecklenburg and Elke Holinski-Feder and Verena Steinke-Lange and Jukka-Pekka Mecklin and John-Paul Plazzer and Marta Pineda and Matilde Navarro and Vidal, \{Joan Brunet\} and Revital Kariv and Guy Rosner and Pi{\~n}ero, \{Tamara Alejandra\} and Gonzalez, \{Mar{\'i}a Laura\} and Pablo Kalfayan and Sampson, \{Julian R\} and Ryan, \{Neil A J\} and Evans, \{D Gareth\} and P{\aa}l M{\o}ller and Crosbie, \{Emma J\}",

year = "2020",

month = jul,

day = "18",

doi = "10.3390/jcm9072290",

language = "English",

volume = "9",

journal = "Journal of Clinical Medicine",

issn = "2077-0383",

publisher = "MDPI",

number = "7",

}

Dominguez-Valentin, M, Seppälä, TT, Engel, C, Aretz, S, Macrae, F, Winship, I, Capella, G, Thomas, H, Hovig, E, Nielsen, M, Sijmons, RH, Bertario, L, Bonanni, B, Tibiletti, MG, Cavestro, GM, Mints, M, Gluck, N, Katz, L, Heinimann, K, Vaccaro, CA, Green, K, Lalloo, F, Hill, J, Schmiegel, W, Vangala, D, Perne, C, Strauß, H-G, Tecklenburg, J, Holinski-Feder, E, Steinke-Lange, V, Mecklin, J-P, Plazzer, J-P, Pineda, M, Navarro, M, Vidal, JB, Kariv, R, Rosner, G, Piñero, TA, Gonzalez, ML, Kalfayan, P, Sampson, JR, Ryan, NAJ, Evans, DG, Møller, P & Crosbie, EJ 2020, 'Risk-Reducing Gynecological Surgery in Lynch Syndrome: Results of an International Survey from the Prospective Lynch Syndrome Database', Journal of Clinical Medicine, vol. 9, no. 7. https://doi.org/10.3390/jcm9072290

TY - JOUR

T1 - Risk-Reducing Gynecological Surgery in Lynch Syndrome

T2 - Results of an International Survey from the Prospective Lynch Syndrome Database

AU - Dominguez-Valentin, Mev

AU - Seppälä, Toni T

AU - Engel, Christoph

AU - Aretz, Stefan

AU - Macrae, Finlay

AU - Winship, Ingrid

AU - Capella, Gabriel

AU - Thomas, Huw

AU - Hovig, Eivind

AU - Nielsen, Maartje

AU - Sijmons, Rolf H

AU - Bertario, Lucio

AU - Bonanni, Bernardo

AU - Tibiletti, Maria Grazia

AU - Cavestro, Giulia Martina

AU - Mints, Miriam

AU - Gluck, Nathan

AU - Katz, Lior

AU - Heinimann, Karl

AU - Vaccaro, Carlos A

AU - Green, Kate

AU - Lalloo, Fiona

AU - Hill, James

AU - Schmiegel, Wolff

AU - Vangala, Deepak

AU - Perne, Claudia

AU - Strauß, Hans-Georg

AU - Tecklenburg, Johanna

AU - Holinski-Feder, Elke

AU - Steinke-Lange, Verena

AU - Mecklin, Jukka-Pekka

AU - Plazzer, John-Paul

AU - Pineda, Marta

AU - Navarro, Matilde

AU - Vidal, Joan Brunet

AU - Kariv, Revital

AU - Rosner, Guy

AU - Piñero, Tamara Alejandra

AU - Gonzalez, María Laura

AU - Kalfayan, Pablo

AU - Sampson, Julian R

AU - Ryan, Neil A J

AU - Evans, D Gareth

AU - Møller, Pål

AU - Crosbie, Emma J

PY - 2020/7/18

Y1 - 2020/7/18

N2 - 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.

AB - 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.

U2 - 10.3390/jcm9072290

DO - 10.3390/jcm9072290

M3 - Article (Academic Journal)

C2 - 32708519

SN - 2077-0383

VL - 9

JO - Journal of Clinical Medicine

JF - Journal of Clinical Medicine

IS - 7

ER -

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

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