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Hum Pathol. 2015 Jan;46(1):153-8. doi: 10.1016/j.humpath.2014.09.015. Epub 2014 Oct 18.

Low-grade small round cell tumor of the cauda equina with EWSR1-WT1 fusion and indolent clinical course.

Author information

1
Department of Pathology and Microbiology, Section of Histopathology, Aga Khan University Hospital, Karachi Pakistan. Electronic address: nasir.uddin@aku.edu.
2
Department of Pathology, University of California San Francisco (UCSF), San Francisco, CA, 94143.
3
Department of Neurosurgery, Aga Khan University Hospital, Karachi Pakistan.
4
Department of Pathology and Microbiology, Section of Histopathology, Aga Khan University Hospital, Karachi Pakistan.
5
Departments of Pathology and Pediatrics, Texas Children's Hospital and Baylor College of Medicine, Houston, TX, 77030.
6
Department of Pathology, University of California San Francisco (UCSF), San Francisco, CA, 94143; Department of Neurological Surgery, University of California San Francisco, San Francisco, CA, 94143.

Abstract

We report a case of a longstanding, large tumor involving spinal nerve roots of the cauda equina. The tumor showed small round cells arranged in nests and cords and immunophenotypic features of a glomus tumor, along with infrequent mitoses and a low Ki-67 labeling index, but exhibited some rosette-like structures, with focal CD99 and Neu-N expression. Subsequent molecular analysis showed the presence of an EWSR1-WT1 gene fusion by fluorescence in situ hybridization, which was confirmed by reverse- transcriptase polymerase chain reaction. To our knowledge, this is the first case reported with EWSR1-WT1 fusion in a small round blue cell tumor with smooth muscle differentiation and an indolent course.

KEYWORDS:

Bilateral; Desmoplastic small round cell tumor; EWSR1; EWSR1-WT1; Glomus tumor; Spinal nerves

PMID:
25454478
DOI:
10.1016/j.humpath.2014.09.015
[Indexed for MEDLINE]

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