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Primary Sclerosing Epithelioid Fibrosarcoma of the Lung in a Patient with Lynch Syndrome

Leisibach, Priska ; Weder, Walter ; Soltermann, Alex ; Jungraithmayr, Wolfgang

In: Lung, 2012, vol. 190, no. 6, p. 691-695

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    Titre
    • Primary Sclerosing Epithelioid Fibrosarcoma of the Lung in a Patient with Lynch Syndrome
    Auteur
    • Leisibach, Priska. Division of Thoracic Surgery, University Hospital Zurich, Raemistr. 100, 8091, Zurich, Switzerland
    • Weder, Walter. Division of Thoracic Surgery, University Hospital Zurich, Raemistr. 100, 8091, Zurich, Switzerland
    • Soltermann, Alex. Institute for Surgical Pathology, University Hospital Zurich, Schmelzbergstr. 12, 8091, Zurich, Switzerland
    • Jungraithmayr, Wolfgang. Division of Thoracic Surgery, University Hospital Zurich, Raemistr. 100, 8091, Zurich, Switzerland
    Type de document
    • Postprint
    Identifiant OAI-PMH
    • oai:doc.rero.ch:320514
    Summary
    Sclerosing epithelioid fibrosarcoma (SEF) is a rare neoplasm arising mostly in limbs and limb girdles, with a high rate of recurrence and a strong tendency to metastasize. This case study is of a 54-year-old woman with an asymptomatic mass in the upper lobe of the left lung detected by PET-CT when staging for Lynch syndrome-associated colon carcinoma. Histology of the resected tumor showed epithelioid cells arranged in nests, partly restiform within a zone of sclerosing fibrosis. Immunohistochemistry was positive for vimentin, epithelial membrane antigen, and S100-protein. Eight months after lung resection, the patient was diagnosed for basal cell carcinoma on her back. At the end of a twoyear follow-up period, she developed metastases to the mediastinum, vertebrae, ribs, femurs, pelvic bones, kidneys, and one lung, histologically all related to SEF. Here we report the first case of a SEF primarily arising from the lung and discuss it in the context of the current literature