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  • A Case of Peutz-Jeghers Syndrome With Breast Cancer, Bilateral Sex Cord Tumor With Annular Tubules, and Adenoma Malignum Caused by STK11 Gene Mutation

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Breast Cancer
A Case of Peutz-Jeghers Syndrome With Breast Cancer, Bilateral Sex Cord Tumor With Annular Tubules, and Adenoma Malignum Caused by STK11 Gene Mutation
  1. Aine Clements, MD*,
  2. Katina Robison, MD*,
  3. Cornelius Granai, MD*,
  4. Margaret M. Steinhoff, MD†,
  5. Jennifer Scalia-Wilbur, MD* and
  6. Richard G. Moore, MD*
  1. * Program in Women's Oncology, Department of Obstetrics and Gynecology, and
  2. † Department of Pathology, Women and Infants Hospital, Brown University, Providence, RI.
  1. Address correspondence and reprint requests to Richard G. Moore, MD, Program in Women's Oncology, Department of Obstetrics and Gynecology, Women and Infants Hospital, Brown University, Providence, RI. E-mail: rmoore@wihri.org.

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.

  • Peutz-Jeghers syndrome
  • Adenoma malignum
  • Sex cord stromal tumors with annular tubules
  • Breast cancer

https://doi.org/10.1111/IGC.0b013e3181ae3f71

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