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

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  • Additional Information
    • Transliterated Title:
      Das un- und dedifferenzierte Endometriumkarzinom : Eine seltene Entität mit breitem differenzialdiagnostischem Spektrum.
    • Source:
      Publisher: Springer-Verlag Country of Publication: Germany NLM ID: 8006541 Publication Model: Print Cited Medium: Internet ISSN: 1432-1963 (Electronic) Linking ISSN: 01728113 NLM ISO Abbreviation: Pathologe Subsets: MEDLINE
    • Publication Information:
      Original Publication: Berlin, New York, Springer-Verlag.
    • Subject Terms:
      Carcinoma, Endometrioid*/diagnosis ; Carcinoma, Endometrioid*/pathology ; Carcinosarcoma*/diagnosis ; Carcinosarcoma*/pathology ; Endometrial Neoplasms*/diagnosis ; Endometrial Neoplasms*/pathology; Biomarkers, Tumor ; Diagnosis, Differential ; Female ; Humans ; Immunohistochemistry
    • 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.
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    • Contributed Indexing:
      Keywords: Cadherins; DNA mismatch repair; Differential diagnosis; Endometrioid carcinoma; Human SMACB1 protein
    • Accession Number:
      0 (Biomarkers, Tumor)
    • Publication Date:
      Date Created: 20191004 Date Completed: 20191120 Latest Revision: 20200108
    • Publication Date:
      20240829
    • Accession Number:
      10.1007/s00292-019-00670-1
    • Accession Number:
      31578630