title: Primary sclerosing epithelioid fibrosarcoma of the lung in a patient with Lynch syndrome.
abstract: 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 two year 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.
authors: Division of Thoracic Surgery, University Hospital Zurich, Raemistr. 100, 8091, Zurich, Switzerland.
authors: Priska
authors: P
authors: Leisibach
authors: Walter
authors: W
authors: Weder
authors: Alex
authors: A
authors: Soltermann
authors: Wolfgang
authors: W
authors: Jungraithmayr
mesh_terms: D003123:Colorectal Neoplasms, Hereditary Nonpolyposis / Q000175:diagnosis*; D005260:Female; D005354:Fibrosarcoma / Q000175:diagnosis* / Q000378:metabolism*; D006801:Humans; D007150:Immunohistochemistry; D008175:Lung Neoplasms / Q000175:diagnosis* / Q000378:metabolism / Q000473:pathology; D008875:Middle Aged; D064847:Multimodal Imaging; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis*; D049268:Positron-Emission Tomography; D012598:Sclerosis; D014057:Tomography, X-Ray Computed