Cytology of Sclerosing Epithelioid Fibrosarcoma in Pleural Effusion
Department of Clinical Laboratory, Seirei Hamamatsu General Hospital, Japan.Diagnostic Cytopathology (Impact Factor: 1.12). 10/2010; 38(10):748-53. DOI: 10.1002/dc.21310
We report a case of sclerosing epithelioid fibrosarcoma focusing on its cytological features in pleural effusion. A 32-year-old man had noticed a tumor in his left buttock 5 years earlier but had not sought treatment because the tumor had been painless. He visited our hospital because the tumor had gradually increased in size. The resected tumor was 12 × 8 × 6 cm in size and had a delineated margin. The histological diagnosis was sclerosing epithelioid fibrosarcoma with a negative surgical margin. Multiple tumor nodules were recognized in both lungs 9 months after the initial surgery, and an excisional biopsy was performed. A histological examination revealed a metastasis of sclerosing epithelioid fibrosarcoma, and chemotherapy was initiated. Pleural disseminations were detected 4 years after the chemotherapy, and the pleural effusion was sampled for cytological examination. The cytological examination revealed several medium-sized cell clusters with moderate overcrowding and an epithelioid cell arrangement; numerous histiocytes and lymphocytes and a small amount of mesothelial cells were observed in the background. The nuclei were pleomorphic with oval, spindle, or cleaved shapes and occasional multinucleation; they were located eccentrically in the cytoplasm and exhibited uniformly fine granular chromatin, a thin nuclear membrane, and several small nucleoli. The above cytological features, coupled with the clinical findings, enabled a diagnosis of sclerosing epithelioid fibrosarcoma. To our knowledge, this is the first cytological description of sclerosing epithelioid fibrosarcoma.
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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.
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