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Primary and metastatic high-grade pleomorphic sarcoma/malignant fibrous histiocytoma of the gastrointestinal tract: an approach to the differential diagnosis in a series of five cases with emphasis on myofibroblastic differentiation

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Abstract

Primary and metastatic so-called malignant fibrous histiocytoma/undifferentiated high-grade pleomorphic sarcoma (MFH) is rare in the gastrointestinal (GI) tract with ≈50 primary and five metastatic cases reported so far. We evaluated two primary gastric and three metastatic intestinal high-grade pleomorphic sarcomas with features of storiform-pleomorphic MFH. Gastric tumours occurred in a 79-year-old man and a 68-year-old woman. One patient died post-operatively, and the other was disease-free at 6 months. Three patients presented with GI metastasis 24, 60 and 0 months after diagnosis of MFH of the heart (n = 1) and the thigh (n = 2). Metastases were located in the small (n = 1) and large bowel (n = 2) and were characteristically pedunculated and polypoid with oedematous haemorrhagic stroma. Concurrent metastases (brain, lung, bone) were present in all three cases. Tumours expressed α-smooth muscle actin (four of five), platelet-derived growth factor receptor (PDGFR) α (three of three) and PDGFRβ (two of three) but were negative for CD117, CD34 and other lineage-specific markers. Ultrastructural examination revealed myo/fibroblastic features. Both gastric MFH were wild type for KIT and PDGFRα. In conclusion, primary and metastatic MFH of the GI tract commonly express PDGFRα and show a myo/fibroblastic phenotype. They should be distinguished from a variety of primary and metastatic pleomorphic neoplasms, in particular high-grade sarcomatous GI stromal tumours (GIST), pleomorphic leiomyosarcoma, sarcomatoid carcinoma and other mimics.

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Authors and Affiliations

  1. Institute of Pathology, Klinikum Nürnberg, Prof.-Ernst-Nathan-Strasse 1, 90419, Nürnberg, Germany

    Abbas Agaimy & Peter H. Wünsch

  2. Institute of Pathology, University of Regensburg, Regensburg, Germany

    Andreas Gaumann, Josef Schroeder, Wolfgang Dietmaier, Arndt Hartmann & Ferdinand Hofstaedter

  3. Dermatopathologische Gemeinschaftspraxis, Friedrichshafen, Germany

    Thomas Mentzel

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  1. Abbas Agaimy
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Correspondence to Abbas Agaimy.

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Agaimy, A., Gaumann, A., Schroeder, J. et al. Primary and metastatic high-grade pleomorphic sarcoma/malignant fibrous histiocytoma of the gastrointestinal tract: an approach to the differential diagnosis in a series of five cases with emphasis on myofibroblastic differentiation. Virchows Arch 451, 949–957 (2007). https://doi.org/10.1007/s00428-007-0495-3

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