Endometrial Carcinomas with Intestinal-Type Metaplasia/Differentiation: Does Mismatch Repair System Defects Matter? Case Report and Systematic Review of the Literature.

Laura Ardighieri,Mattia Bugatti,Andrea Palicelli,Federico Ferrari,Emma Drera,Enrico Sartori,Franco Odicino

doi:10.3390/jcm9082552

Abstract

Background: Intestinal metaplasia/differentiation in primary endometrial carcinomas is an uncommon phenomenon, with only few cases described. Material and Methods: We performed a systematic review of endometrial carcinomas with intestinal metaplasia/differentiation interrogating the electronic databases Pubmed, Web of Science, and Scopus, and we reported an additional case arising in a 49-year-old woman. Results: We identified only eight patients diagnosed with endometrial carcinomas exhibiting intestinal metaplasia/differentiation, and additionally our case. Endometrial carcinomas with intestinal-type features can present in pure or mixed forms in association with usual-type endometrioid carcinomas; in mixed forms, the two neoplastic components may derive from a common neoplastic progenitor, as evidenced by the concomitant loss of MSH2 and MSH6 protein expression in our case. Disease recurrences occur in a significant fraction of the cases, including patients diagnosed in low-stage disease. Conclusions: Endometrial carcinomas with intestinal metaplasia/differentiation are rare and they may represent a more aggressive tumor variant, thus requiring a proper treatment despite the low-tumor stage. The ProMise classification should be performed also in these unusual tumors, since they can be associated with mismatch repair system defects.

Highlights

Endometrioid carcinoma is the most common endometrial cancer, often showing a wide spectrum of morphological variants and metaplastic changes that can make the diagnosis challenging [1,2,3]
Material and Methods: We performed a systematic review of endometrial carcinomas with intestinal metaplasia/differentiation interrogating the electronic databases Pubmed, Web of Science, and Scopus, and we reported an additional case arising in a 49-year-old woman
Mucinous differentiation in endometrioid carcinomas is a frequent phenomenon; according to the criteria first proposed by Ross in 1983 [4] and subsequently adopted by the World Health Organization (WHO) classification [1], only endometrial carcinomas (ECs) composed of >50% by mucinous cells are classified as mucinous carcinomas

Summary

Introduction

Endometrioid carcinoma is the most common endometrial cancer, often showing a wide spectrum of morphological variants and metaplastic changes that can make the diagnosis challenging [1,2,3]. Primary mucinous carcinomas of the endometrium comprise 1–9% of all ECs, usually showing an endocervical-type differentiation [5,6]. They are typically well-differentiated with a relatively good prognosis; aggressive cases have been reported [1,7]. Rare variants of mucinous differentiation were described in ECs, including gastric-type and intestinal-type [8,9]. Gastric (gastrointestinal)-type mucinous carcinomas represent a rare and aggressive subtype of EC with specific morphological and immunohistochemical features, including the absence of an endometrioid component [8]. The ProMise classification should be performed in these unusual tumors, since they can be associated with mismatch repair system defects

Methods

Results

Conclusion