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Myoepithelial carcinoma of the paracecal mesentery: aggressive behavior of a rare neoplasm at an unusual anatomic site

Khin Thway, Jonathan Noujaim, D. Michael Thomas, Cyril Fisher, Robin L. Jones
  • Khin Thway
    Sarcoma Unit, Royal Marsden NHS Foundation Trust, London, United Kingdom | khin_thway@yahoo.com
  • Jonathan Noujaim
    Sarcoma Unit, Royal Marsden NHS Foundation Trust, London, United Kingdom
  • D. Michael Thomas
    Department of Cellular Pathology, Maidstone and Tunbridge Wells NHS Trust, Kent, United Kingdom
  • Cyril Fisher
    Sarcoma Unit, Royal Marsden NHS Foundation Trust, London, United Kingdom
  • Robin L. Jones
    Sarcoma Unit, Royal Marsden NHS Foundation Trust, London, United Kingdom

Abstract

Myoepithelial tumors of the soft tissues represent a rare group of neoplasms that vary in their clinical behavior, pathologic features and genetics. They are histopathologically typified by a myoepithelial immunohistochemical phenotype, of expression of one or more epithelial markers, S100 protein and smooth muscle actin. Because of their rarity and occurrence over a wide age range and at a variety of anatomic sites, they can be difficult to diagnose due to the lack of familiarity by physicians, which is compounded by their spectrum of histologic features and morphologic overlap with several other neoplasms. Recent genetic insights have aided classification, and it is increasingly understood that soft tissue myoepithelial neoplasms can be stratified into two distinct morphologic and genetic subgroups. We describe a case of a 44-year-old man who was diagnosed with a primary myoepithelial neoplasm of the paracecal mesentery, which showed aggressive local recurrence after four years. The tumor was composed of cords of ovoid cells within chondromyxoid stroma, and displayed a characteristic pancytokeratin, S100 protein and smooth muscle actin-positive myoepithelial immunoprofile. Primary myoepithelioma has not been previously described at this site, and this case highlights this varied family of tumors, emphasizes the need to consider myoepithelial tumor in the differential diagnoses of carcinoma variants occurring in the bowel or mesentery, and also adds to the number of reported myoepithelial neoplasms showing markedly aggressive behavior.

Keywords

Myoepithelial tumor; myoepithelial carcinoma; myoepithelioma; mixed tumor; peritoneum; bowel; cecum; EWSR1; metastasis; soft tissue

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Submitted: 2016-03-12 21:20:22
Published: 2017-03-31 08:43:13
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Copyright (c) 2017 Khin Thway, Jonathan Noujaim, D Michael Thomas, Cyril Fisher, Robin L Jones

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