A Case of Peutz-Jeghers Syndrome With Breast Cancer, Bilateral Sex Cord Tumor With Annular Tubules, and Adenoma Malignum Caused by STK11 Gene Mutation. Issue 9 (1st November 2009)

• Record Type: Journal Article
• Authors: Clements, Aine; Robison, Katina; Granai, Cornelius; Steinhoff, Margaret M.; Scalia-Wilbur, Jennifer; Moore, Richard G.
• Is Part Of: International journal of gynecological cancer. Volume 19:Issue 9(2009)
• Page Start: 1591
• Page End: 1594
• Publication Date: 2009-11-01
• Subjects: Peutz-Jeghers syndrome -- Adenoma malignum -- Sex cord stromal tumors with annular tubules -- Breast cancer; Generative organs, Female -- Cancer -- Periodicals; 616.99465
• DOI: 10.1111/IGC.0b013e3181ae3f71
• Languages: English
• ISSNs: 1048-891X

Abstract

Abstract : 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.