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Uptake of hysterectomy and bilateral salpingo-oophorectomy in carriers of pathogenic mismatch repair variants: a Prospective Lynch Syndrome Database report.


ABSTRACT:

Purpose

This study aimed to report the uptake of hysterectomy and/or bilateral salpingo-oophorectomy (BSO) to prevent gynaecological cancers (risk-reducing surgery [RRS]) in carriers of pathogenic MMR (path_MMR) variants.

Methods

The Prospective Lynch Syndrome Database (PLSD) was used to investigate RRS by a cross-sectional study in 2292 female path_MMR carriers aged 30-69 years.

Results

Overall, 144, 79, and 517 carriers underwent risk-reducing hysterectomy, BSO, or both combined, respectively. Two-thirds of procedures before 50 years of age were combined hysterectomy and BSO, and 81% of all procedures included BSO. Risk-reducing hysterectomy was performed before age 50 years in 28%, 25%, 15%, and 9%, and BSO in 26%, 25%, 14% and 13% of path_MLH1, path_MSH2, path_MSH6, and path_PMS2 carriers, respectively. Before 50 years of age, 107 of 188 (57%) BSO and 126 of 204 (62%) hysterectomies were performed in women without any prior cancer, and only 5% (20/392) were performed simultaneously with colorectal cancer (CRC) surgery.

Conclusion

Uptake of RRS before 50 years of age was low, and RRS was rarely undertaken in association with surgical treatment of CRC. Uptake of RRS aligned poorly with gene- and age-associated risk estimates for endometrial or ovarian cancer that were published recently from PLSD and did not correspond well with current clinical guidelines. The reasons should be clarified. Decision-making on opting for or against RRS and its timing should be better aligned with predicted risk and mortality for endometrial and ovarian cancer in Lynch syndrome to improve outcomes.

SUBMITTER: Seppala TT 

PROVIDER: S-EPMC8916840 | biostudies-literature | 2021 May

REPOSITORIES: biostudies-literature

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Publications

Uptake of hysterectomy and bilateral salpingo-oophorectomy in carriers of pathogenic mismatch repair variants: a Prospective Lynch Syndrome Database report.

Seppälä Toni T TT   Dominguez-Valentin Mev M   Crosbie Emma J EJ   Engel Christoph C   Aretz Stefan S   Macrae Finlay F   Winship Ingrid I   Capella Gabriel G   Thomas Huw H   Hovig Eivind E   Nielsen Maartje M   Sijmons Rolf H RH   Bertario Lucio L   Bonanni Bernardo B   Tibiletti Maria G MG   Cavestro Giulia M GM   Mints Miriam M   Gluck Nathan N   Katz Lior L   Heinimann Karl K   Vaccaro Carlos A CA   Green Kate K   Lalloo Fiona F   Hill James J   Schmiegel Wolff W   Vangala Deepak D   Perne Claudia C   Strauß Hans-Georg HG   Tecklenburg Johanna J   Holinski-Feder Elke E   Steinke-Lange Verena V   Mecklin Jukka-Pekka JP   Plazzer John-Paul JP   Pineda Marta M   Navarro Matilde M   Vida Joan B JB   Kariv Revital R   Rosner Guy G   Piñero Tamara A TA   Pavicic Walter W   Kalfayan Pablo P   Ten Broeke Sanne W SW   Jenkins Mark A MA   Sunde Lone L   Bernstein Inge I   Burn John J   Greenblatt Marc M   de Vos Tot Nederveen Cappel Wouter H WH   Della Valle Adriana A   Lopez-Koestner Francisco F   Alvarez Karin K   Büttner Reinhard R   Görgens Heike H   Morak Monika M   Holzapfel Stefanie S   Hüneburg Robert R   von Knebel Doeberitz Magnus M   Loeffler Markus M   Redler Silke S   Weitz Jürgen J   Pylvänäinen Kirsi K   Renkonen-Sinisalo Laura L   Lepistö Anna A   Hopper John L JL   Win Aung K AK   Lindor Noralane M NM   Gallinger Steven S   Le Marchand Loïc L   Newcomb Polly A PA   Figueiredo Jane C JC   Thibodeau Stephen N SN   Therkildsen Christina C   Wadt Karin A W KAW   Mourits Marian J E MJE   Ketabi Zohreh Z   Denton Oliver G OG   Rødland Einar A EA   Vasen Hans H   Neffa Florencia F   Esperon Patricia P   Tjandra Douglas D   Möslein Gabriela G   Rokkones Erik E   Sampson Julian R JR   Evans D G DG   Møller Pål P  

European journal of cancer (Oxford, England : 1990) 20210317


<h4>Purpose</h4>This study aimed to report the uptake of hysterectomy and/or bilateral salpingo-oophorectomy (BSO) to prevent gynaecological cancers (risk-reducing surgery [RRS]) in carriers of pathogenic MMR (path_MMR) variants.<h4>Methods</h4>The Prospective Lynch Syndrome Database (PLSD) was used to investigate RRS by a cross-sectional study in 2292 female path_MMR carriers aged 30-69 years.<h4>Results</h4>Overall, 144, 79, and 517 carriers underwent risk-reducing hysterectomy, BSO, or both c  ...[more]

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