LAUSR

PeutzJeghers syndrome is an autosomal dominant cancer predisposition syndrome characterized by mutations in the STK11 gene. Gynecologic manifestations of this syndrome include cervical gastrictype mucinous adenocarcinoma, lobular endocervical glandular hyperplasia, sex cord tumor with annular tubules, and less commonly, Sertoli cell tumors. PeutzJeghers syndrome is implicated in up to 10% of cervical gastrictype adenocarcinomas and 36% of sex cord tumors with annular tubules. Here we describe three gynecologic entities seen in a 32yearold woman with PeutzJeghers syndrome presenting with amenorrhea. Cervical biopsy showed gastrictype mucinous adenocarcinoma, prompting a hysterectomy. Intraoperative evaluation of the slightly enlarged ovary (2.5 cm) showed a sex cord tumor with annular tubules, thus, left oophorectomy was also performed. Final histology showed gastrictype mucinous adenocarcinoma, lobular endocervical glandular hyperplasia, and ovarian sex cord tumor with annular tubules. GASTRIC-TYPE ADENOCARCINOMA OF CERVIX Gastrictype mucinous adenocarcinomas are rare, human papillomavirus (HPV)independent adenocarcinomas with a more aggressive behavior than conventional HPVassociated adenocarcinomas despite their deceptively bland morphology. Due to the presence of welldifferentiated mucinous glands, this tumor was previously called ‘minimal deviation adenocarcinoma’ and ‘adenoma malignum’— neither of these terms is currently recommended by the World Health Organization (WHO). Although they can be seen in PeutzJeghers syndrome, these tumors are usually sporadic. The mean patient age is 50–55 years, but they can occur at younger ages when associated with the syndrome. These tumors typically measure 2–5 cm and can often present at late stages with unusual metastases. Grossly, the cervix is either unremarkable or hypertrophic and/ or indurated with multiple cystic lesions. Histology shows welldifferentiated glands (Figure 1A), that may be arranged in papillary architecture (Figure 1B),