Mesonephric-like Adenocarcinoma of the Female Genital Tract: From Morphologic Observations to a Well-characterized Carcinoma With Aggressive Clinical Behavior

Abstract

Mesonephric-like adenocarcinoma (MLA) was introduced as a new tumor type in the endometrium and the ovary in the 2020 World Health Organization (WHO) Classification. This is a rare recently described (2016) and clinically aggressive carcinoma with a propensity for distant spread, especially to the lungs. MLA has a characteristic morphology and immunophenotype (hormone receptor negative; TTF1 and/or GATA3 positive). These neoplasms are commonly associated with KRAS and PIK3CA mutations and in the Cancer Genome Atlas (TCGA) molecular classification of endometrial carcinomas fall into the copy number low/no specific molecular profile category. Although they show significant morphological, immunophenotypic and molecular overlap with cervical mesonephric adenocarcinomas, there are other parameters which suggest a Mullerian origin and, as such, the term MLA seems apt. MLA can be added to the list of endometriosis-associated ovarian neoplasms. In this paper, I outline the series of events which lead to the first description of MLA and review the subsequent literature on this tumor type which has expanded on the morphologic features and immunophenotype, discovered the molecular underpinnings and elucidated the clinical behavior. The discovery of MLA represents an example of “new” entities still to this day being discovered through careful morphologic observations and referral of cases for specialist opinion.