Un- and dedifferentiated endometrial carcinoma A rare entity with a wide range of differential diagnosis

Hoehn AK, Brambs CE, Opitz S, Erber R, Hartmann A, Horn LC (2019)

Publication Type: Journal article

Publication year: 2019

Journal

Pathologe Springer Verlag (Germany)

DOI: 10.1007/s00292-019-00670-1

Abstract

Dedifferentiated endometrial carcinomas (ECs) are composed of undifferentiated EC and a FIGO grade 1 or 2 endometrioid carcinoma. The undifferentiated component represents a malignant epithelial neoplasm with no obvious differentiation and immunohistochemical loss of PAX8, E-cadherin and focal expression of EMA and/or CK18 and the predominant presence of nuclear staining for INI1 (SMARCB1) and BRG1 (SMARCA4). The main differential diagnoses include poorly differentiated endometrioid EC, neuroendocrine carcinoma, lymphoma, plasmocytoma, high-grade endometrial stromal sarcomas, undifferentiated uterine sarcomas (UUS), carcinosarcomas, and metastases to the endometrium. The histogenesis is not yet fully understood and molecular data are still limited. Some tumors represent a loss of MHL1 and PMS2 staining due to MLH1-promotor methylation. Rare cases are associated with Lynch syndrome or POLE mutation. The un- or dedifferentiated EC represents a high-grade endometrial carcinoma that requires extended surgery and indicates a poor prognosis. In cases with mismatch repair protein deficiency or POLE mutation, immuno-oncological treatment with checkpoint inhibitors are a therapeutic option.

Authors with CRIS profile

Ramona Erber Institute of Pathology Arndt Hartmann Lehrstuhl für Allgemeine Pathologie und Pathologische Anatomie

Involved external institutions

Universitätsklinikum Leipzig DE Germany (DE) Technische Universität München (TUM) DE Germany (DE)

How to cite

APA:

Hoehn, A.K., Brambs, C.E., Opitz, S., Erber, R., Hartmann, A., & Horn, L.-C. (2019). Un- and dedifferentiated endometrial carcinoma A rare entity with a wide range of differential diagnosis. Pathologe. https://doi.org/10.1007/s00292-019-00670-1

MLA:

Hoehn, A. K., et al. "Un- and dedifferentiated endometrial carcinoma A rare entity with a wide range of differential diagnosis." Pathologe (2019).

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