Primary renal sclerosing epithelioid fibrosarcoma

Report of 2 cases with EWSR1-CREB3L1 gene fusion

Pedram Argani, Jack R. Lewin, Pamela Edmonds, George J. Netto, Carlos Prieto-Granada, Lei Zhang, Achim A. Jungbluth, Cristina R. Antonescu

Research output: Contribution to journalArticle

Abstract

We report the first 2 genetically confirmed cases of primary renal sclerosing epithelioid fibrosarcoma (SEF), occurring in a 17-year-old boy and a 61-year-old woman. In both cases, the tumors demonstrated the typical epithelioid clear cell morphology associated with extensive hyalinizing fibrosis, raising the differential diagnosis of solitary fibrous tumor, metanephric stromal tumor, and the sclerosing variant of clear cell sarcoma of the kidney. Both neoplasms demonstrated diffuse immunoreactivity for MUC4, a highly specific marker for SEF, and both demonstrated evidence of rearrangement of both the EWSR1 and CREB3L1 genes, which have recently been shown to be fused in this entity. Both neoplasms presented with metastatic disease. Primary renal SEF represents yet another translocation-associated sarcoma now shown to arise primarily in the kidney.

Original languageEnglish (US)
Pages (from-to)365-373
Number of pages9
JournalAmerican Journal of Surgical Pathology
Volume39
Issue number3
DOIs
StatePublished - Feb 28 2015

Fingerprint

Fibrosarcoma
Gene Fusion
Kidney
Neoplasms
Clear Cell Sarcoma
Solitary Fibrous Tumors
Epithelioid Cells
Sarcoma
Differential Diagnosis
Fibrosis
Genes

Keywords

  • fibrosarcoma
  • renal neoplasm
  • translocation

ASJC Scopus subject areas

  • Anatomy
  • Pathology and Forensic Medicine
  • Surgery

Cite this

Primary renal sclerosing epithelioid fibrosarcoma : Report of 2 cases with EWSR1-CREB3L1 gene fusion. / Argani, Pedram; Lewin, Jack R.; Edmonds, Pamela; Netto, George J.; Prieto-Granada, Carlos; Zhang, Lei; Jungbluth, Achim A.; Antonescu, Cristina R.

In: American Journal of Surgical Pathology, Vol. 39, No. 3, 28.02.2015, p. 365-373.

Research output: Contribution to journalArticle

Argani, P, Lewin, JR, Edmonds, P, Netto, GJ, Prieto-Granada, C, Zhang, L, Jungbluth, AA & Antonescu, CR 2015, 'Primary renal sclerosing epithelioid fibrosarcoma: Report of 2 cases with EWSR1-CREB3L1 gene fusion', American Journal of Surgical Pathology, vol. 39, no. 3, pp. 365-373. https://doi.org/10.1097/PAS.0000000000000338
Argani, Pedram ; Lewin, Jack R. ; Edmonds, Pamela ; Netto, George J. ; Prieto-Granada, Carlos ; Zhang, Lei ; Jungbluth, Achim A. ; Antonescu, Cristina R. / Primary renal sclerosing epithelioid fibrosarcoma : Report of 2 cases with EWSR1-CREB3L1 gene fusion. In: American Journal of Surgical Pathology. 2015 ; Vol. 39, No. 3. pp. 365-373.
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