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.
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ABSTRACT: Sclerosing epithelioid fibrosarcoma (SEF) is a clinicopathologically distinct variant of fibrosarcoma that is capable of recurrence and metastasis. Awareness of imaging features and histopathologic characteristics will be helpful for differential diagnosis from other common tumors. Here, we report a case of SEF metastasizing to the pancreas as a solitary mass mimicking primary pancreatic cancer, and summarize the reported cases with FDG PET/CT from the literature (n=4). PET/CT showed abnormal FDG accumulation (n=2), mild FDG uptake (n=1), or photopenic (n=1). The FDG PET/CT features are closely related to histopathologic characteristics regarding its differentiation and aggressiveness.
The Journal of Dermatology 06/2013; 40(8). DOI:10.1111/1346-8138.12190 · 2.35 Impact Factor