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.
References
Meis-Kindblom JM, Kindblom LG, Enzinger FM (1995) Sclerosing epithelioid fibrosarcoma. A variant of fibrosarcoma simulating carcinoma. Am J Surg Pathol 19(9):979–993
Smith PJ, Almeida B, Krajacevic J, Taylor B (2008) Sclerosing epithelioid fibrosarcoma as a rare cause of ascites in a young man: a case report. J Med Case Rep 2:248
Ossendorf C, Studer GM, Bode B, Fuchs B (2008) Sclerosing epithelioid fibrosarcoma: case presentation and a systematic review. Clin Orthop Relat Res 466(6):1485–1491
Hansen T, Katenkamp K, Brodhun M, Katenkamp D (2006) Low-grade fibrosarcoma—report on 39 not otherwise specified cases and comparison with defined low-grade fibrosarcoma types. Histopathology 49(2):152–160
Tsuchido K, Yamada M, Satou T, Otsuki Y, Shimizu S, Kobayashi H (2010) Cytology of sclerosing epithelioid fibrosarcoma in pleural effusion. Diagn Cytopathol 38(10):748–753
Nilbert M, Therkildsen C, Nissen A, Akerman M, Bernstein I (2009) Sarcomas associated with hereditary nonpolyposis colorectal cancer: broad anatomical and morphological spectrum. Fam Cancer 8(3):209–213
Chang JM, Lee HJ, Goo JM, Lee HY, Lee JJ, Chung JK, Im JG (2006) False positive and false negative FDG-PET scans in various thoracic diseases. Korean J Radiol 7(1):57–69
Yu VP, Novelli M, Payne SJ, Fisher S, Barnetson RA, Frayling IM, Barrett A, Goudie D, Ardern-Jones A, Eeles R, Shanley S (2009) Unusual presentation of Lynch syndrome. Hered Cancer Clin Pract 7(1):12
Koornstra JJ, Mourits MJ, Sijmons RH, Leliveld AM, Hollema H, Kleibeuker JH (2009) Management of extracolonic tumours in patients with Lynch syndrome. Lancet Oncol 10(4):400–408
Heinimann K, Müller H, Weber W, Scott RJ (1997) Disease expression in Swiss hereditary non-polyposis colorectal cancer (HNPCC) kindreds. Int J Cancer 74(3):281–285
Peltomäki P, Gao X, Mecklin JP (2001) Genotype and phenotype in hereditary nonpolyposis colon cancer: a study of families with different vs. shared predisposing mutations. Fam Cancer 1(1):9–15
Nolan L, Eccles D, Cross E, Crawford G, Beck N, Bateman A, Ottensmeier C (2009) First case report of Muir-Torre syndrome associated with non-small cell lung cancer. Fam Cancer 8(4):359–362
Ang JM, Alai NN, Ritter KR, Machtinger LA (2011) Muir-Torre syndrome: case report and review of the literature. Cutis 87(3):125–128
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Leisibach, P., Weder, W., Soltermann, A. et al. Primary Sclerosing Epithelioid Fibrosarcoma of the Lung in a Patient with Lynch Syndrome. Lung 190, 691–695 (2012). https://doi.org/10.1007/s00408-012-9401-0
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DOI: https://doi.org/10.1007/s00408-012-9401-0