PT - JOURNAL ARTICLE AU - Aine Clements AU - Katina Robison AU - Cornelius Granai AU - Margaret M. Steinhoff AU - Jennifer Scalia-Wilbur AU - Richard G. Moore TI - A Case of Peutz-Jeghers Syndrome With Breast Cancer, Bilateral Sex Cord Tumor With Annular Tubules, and Adenoma Malignum Caused by <em>STK11</em> Gene Mutation AID - 10.1111/IGC.0b013e3181ae3f71 DP - 2009 Nov 01 TA - International Journal of Gynecologic Cancer PG - 1591--1594 VI - 19 IP - 9 4099 - http://ijgc.bmj.com/content/19/9/1591.short 4100 - http://ijgc.bmj.com/content/19/9/1591.full SO - Int J Gynecol Cancer2009 Nov 01; 19 AB - Background: Peutz-Jeghers syndrome (PJS) is a rare autosomal dominant disorder, and women with this syndrome are at an increased risk of developing intestinal and extraintestinal malignancies including breast and gynecologic malignancies. This case report presents a patient with PJS with a concomitant breast cancer, bilateral stromal tumors with annular tubules of the ovaries, and adenoma malignum of the cervix.Case: A 43-year-old woman presented with an advanced-stage breast cancer and a pelvic mass. The patient was treated with neoadjuvant chemotherapy followed by laparotomy with a hysterectomy and oophorectomy. Final pathologic examination revealed a concomitant breast cancer with metastasis to the ovaries, bilateral stromal tumors with annular tubules of the ovaries, and adenoma malignum of the cervix.Conclusions: Patients with PJS are at a high risk for intestinal and extraintestinal malignancies and can present with multiple concomitant malignancies.