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Sitzmann JV, Wiebke EA. Risk-Reducing Appendectomy and the Elimination of BRCA1 -Associated Intraperitoneal Cancer. JAMA Surg. 2013;148(3):285–291. doi:10.1001/jamasurg.2013.1006
Author Affiliations: Department of
Surgery, Indiana University School of Medicine, and Surgical Service,
Roudebush Veterans Affairs Medical Center, Indianapolis.
Risk-reducing bilateral salpingo-oophorectomy (RRBSO) and risk-reducing
mastectomy are widely used for BRCA1 and BRCA2 mutation carriers to reduce the risk of ovarian
and breast cancer. To our knowledge, no risk-reduction therapy has
addressed the BCRA1/2 carrier lifetime risk
of intra-abdominal peritoneal carcinoma from an appendix source. We
identified a BRCA1 carrier in a hereditary
breast and ovarian cancer kindred who developed a low-grade malignant
appendiceal mucocele 2 years after risk-reducing salpingo-oophorectomy.
Our retrospective meta-analysis assessed the risk of intraperitoneal
appendiceal cancer in BRCA1/2 carriers after
RRBSO to determine whether elective risk-reduction appendectomy could
reduce the incidence of intraperitoneal cancer. Data sources included
the case report and 12 reports of BRCA1 and BRCA2 carriers after RRBSO with ovarian, fallopian
tube, breast, and peritoneal cancer published from January 1, 1985,
through April 30, 2012. Main outcome measures were nonovarian, non–fallopian
tube, nonbreast, positive intra-abdominal peritoneal carcinoma in
previously cancer-free BRCA1/2 carriers after
RRBSO. The source of intraperitoneal cancer in BRCA1/2 carriers after risk-reducing salpingo-oophorectomy is highly likely
the appendix. Use of risk-reduction appendectomy with RRBSO in younger BRCA1/2 carriers may reduce lifetime risk of malignant
tumor and eliminate intraperitoneal cancer.
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