An unusual admixture of neoplastic and metaplastic lesions of the female genital tract in the Peutz-Jeghers Syndrome

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

“…5 Multiple ovarian neoplasms can be seen affecting an individual patient as described in a patient with PJS with a serous cystadenoma, mixed serous and mucinous low malignant potential tumors, and SCTATs of the ovary. 14 Adenoma malignum (minimal deviation adenocarcinoma) is a rare endocervical columnar adenocarcinoma of the cervix with increased incidence in patients with PJS. These tumors are often well differentiated located in the endocervix and deeply invasive into the cervical stroma.…”

Endometrial metaplasias and reactive changes: a spectrum of altered differentiation

“…Nevertheless, mucinous lesions may represent monoclonal alteration of the endometrium,44 and this would explain their relationship with neoplasia. This is clearly seen in STK-11 gene mutations, especially in patients with Peutz–Jeghers syndrome,45 where EMM occurs as a spectrum of histological changes ranging from metaplasia to carcinoma, involving synchronously cervix, corpus, fallopian tube and ovary. Multifocality, however, can also occur sporadically 41 43…”

Endometrial Intestinal Metaplasia

“…Simultaneous mullerian mucinous lesions in the female genital tract involving the ovarian surface, fallopian tube epithelium, endometrium, and cervix may occur in both PJS and secondary to sporadic mutations of STK11 gene, the gene implicated in PJS (9,10). Furthermore, it is well known that PJS can be associated with endocervical minimal deviation adenocarcinoma (11).…”