STK11 Adnexal Tumor: Exploring the Association With Peutz-Jeghers Syndrome and its Distinction From Morphologic Mimickers

Jennifer A Bennett; Esther Oliva

doi:10.1097/PAP.0000000000000460

STK11 Adnexal Tumor: Exploring the Association With Peutz-Jeghers Syndrome and its Distinction From Morphologic Mimickers

Adv Anat Pathol. 2024 Sep 3. doi: 10.1097/PAP.0000000000000460. Online ahead of print.

Authors

Jennifer A Bennett¹, Esther Oliva²

Affiliations

¹ Department of Pathology, University of Chicago, Chicago, IL.
² Department of Pathology, Massachusetts General Hospital, Boston, MA.

PMID: 39225118
DOI: 10.1097/PAP.0000000000000460

Abstract

STK11 adnexal tumor is a novel malignant neoplasm of uncertain histogenesis frequently arising in a para-adnexal location and associated with Peutz-Jeghers syndrome in ∼50% of patients. Its broad morphologic spectrum and nonspecific immunohistochemical profile has resulted in misclassification in the past as a variety of other neoplasms including those of wolffian, sex cord-stromal, mesothelial, and epithelial derivation. This review focuses on the spectrum of adnexal neoplasms that may develop in Peutz-Jeghers syndrome, with particular emphasis on STK11 adnexal tumor and its differential diagnosis.