Peutz-Jeghers syndrome with gastric-type mucinous endocervical adenocarcinoma and sex-cord tumor with annular tubules: A case report

Xuanyan Li; Yue Qi; Wenwen Zhang; Yang Rao; Na Zhang; Pengpeng Qu

doi:10.3389/fmed.2023.1094839

Peutz-Jeghers syndrome with gastric-type mucinous endocervical adenocarcinoma and sex-cord tumor with annular tubules: A case report

Front Med (Lausanne). 2023 Mar 21:10:1094839. doi: 10.3389/fmed.2023.1094839. eCollection 2023.

Authors

Xuanyan Li^{1

2}, Yue Qi^{1

3}, Wenwen Zhang¹, Yang Rao¹, Na Zhang¹, Pengpeng Qu^{1

2

3}

Affiliations

¹ Department of Gynecological Oncology, Tianjin Central Hospital Gynecology Obstetrics, Tianjin, China.
² Nankai University School of Medicine, Nankai University, Tianjin, China.
³ Clinical College of Central Gynecology and Obstetrics, Tianjin Medical University, Tianjin, China.

Abstract

Peutz-Jeghers syndrome (PJS) is a rare autosomal dominant genetic disorder characterized by mucocutaneous pigmentation and multiple hamartomatous polyps in the gastrointestinal tracts. About 11% of female PJS patients are diagnosed with Gastric-type endocervical adenocarcinoma (G-EAC) and about one third have a sex-cord tumor with annular tubules (SCTATs). Gastric-type endocervical adenocarcinoma is a special subtype of cervical adenocarcinoma which accounts for only 1-3%. Here we report a rare case of a 31-year-old woman affected with G-EAC and SCTAT accompanied by PJS. After surgery, we followed up for 5 years without recurrence.

Keywords: Peutz-Jeghers syndrome; diagnosis; gastric-type mucinous endocervical adenocarcinoma; sex-cord tumor with annular tubules; treatment.

Publication types

Case Reports