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
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.
Topics & Concepts
Peutz–Jeghers syndromeMucocutaneous zoneMedicineAdenocarcinomaSTK11PathologyGastric adenocarcinomaCancerInternal medicineColorectal cancerDiseaseKRASGenetic factors in colorectal cancerMultiple and Secondary Primary CancersCholangiocarcinoma and Gallbladder Cancer Studies