Primary sclerosing epitheloid fibrosarcoma of the kidney: First case reported in Mexico

Here we reported a case of primary sclerosing epitheloid fibrosarcoma (SEF) of the kidney, an extremely rare and aggressive tumor. The patient presented a mass in the upper part of the right kidney with pulmonary metastasis at the time of diagnosis, a right radical nephrectomy was performed, it was...

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Bibliographic Details
Published inUrology case reports Vol. 53; p. 102697
Main Authors Jiménez-López, L.A., Ocampo-Montero, A., Homs Toache, J.R.
Format Journal Article
LanguageEnglish
Published United States Elsevier Inc 01.03.2024
Elsevier
Subjects
CREB3L1
EWSR1
Kidney
MUC4 translocation
Primary sclerosing epitheloid fibrosarcoma
Primary sclerosing epitheloid fibrosarcoma
MUC4 translocation
EWSR1
CREB3L1
Kidney
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Summary:Here we reported a case of primary sclerosing epitheloid fibrosarcoma (SEF) of the kidney, an extremely rare and aggressive tumor. The patient presented a mass in the upper part of the right kidney with pulmonary metastasis at the time of diagnosis, a right radical nephrectomy was performed, it was a solid tumor of 15 cm × 9 cm affecting almost the entire kidney. Histological study revealed a neoplasm of uniform epithelioid cells with scant cytoplasm with dense and sclerotic stroma. immunohistochemistry positive for MUC-4, detection by fusion of EWSR1-CREB3L1 by FISH positive.
ISSN:2214-4420
2214-4420
DOI:10.1016/j.eucr.2024.102697