Sclerosing Epithelioid Fibrosarcoma of the Kidney: Clinicopathologic and Molecular Study of a Rare Neoplasm at a Novel Location

Sclerosing epithelioid fibrosarcoma (SEF) is a rare fibrosarcoma variant with specific histomorphology and consistent translocation (EWSR1-CREB3L1/2). To date, ̴ 110 cases have been reported; only 15 originated within the abdomen. With only 3 cases reported parallel to our study, primary renal SEF are exceptionally rare but might be under-recognized. We herein describe two cases affecting a 23–year-old female and a 43-year-old male. Tumor size was 22 cm and 4.2 cm, respectively. Patient 1 developed skeletal and multiple pulmonary metastases.

Source: Annals of Diagnostic Pathology - May 6, 2015 Category: Pathology Authors: Carsten-Henning Ohlmann, Ines B. Brecht, Kerstin Junker, Jill A. van der Zee, Adriana Nistor, Rainer M. Bohle, Michael Stöckle, Markus Metzler, Arndt Hartmann, Abbas Agaimy Tags: Original Contribution Source Type: research