Desmoplastic small round cell tumor: A rare cause of a progressive brachial plexopathy.

Mathys, Jan; Vajtai, Istvan; Vögelin, Esther; Zimmermann, Dieter R; Ozdoba, Christoph; Hewer, Ekkehard (2014). Desmoplastic small round cell tumor: A rare cause of a progressive brachial plexopathy. Muscle & nerve, 49(6), pp. 922-927. John Wiley & Sons 10.1002/mus.24165

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Introduction: Desmoplastic small round cell tumor (DSRCT) is an uncommon, embryonic-type neoplasm, typically presenting as an abdominal mass in young men. A single case of DSRCT arising in the peripheral nervous system has been reported. Methods: The clinical course, imaging, electrophysiological, intraoperative, histopathological, molecular findings, and postoperative follow-up are reported. Results: A 43-year-old man presented with slowly progressive right brachial plexopathy. Magnetic resonance imaging revealed an enlarged medial cord with heterogeneous contrast enhancement. Histology showed a "small round cell" neoplasm with a polyphenotypic immunoprofile, including epithelial and mesenchymal markers. A pathognomonic fusion of Ewing sarcoma breakpoint region 1 and Wilms tumor 1 genes (EWSR1/WT1) was present. Treatment involved gross total excision and local radiotherapy. Conclusion: Our findings confirm the occurrence of DSRCT as a primary peripheral nerve tumor. Despite its usually very aggressive clinical course, prolonged recurrence-free survival may be reached. Histomorphology and immunoprofile of DSRCT may lead to misdiagnosis as small cell carcinoma. © 2013 Wiley Periodicals, Inc.

Item Type:

Journal Article (Further Contribution)

Division/Institute:

04 Faculty of Medicine > Service Sector > Institute of Pathology > Clinical Pathology
04 Faculty of Medicine > Pre-clinic Human Medicine > BioMedical Research (DBMR) > Forschungsbereich Mu50 > Forschungsgruppe Handchirurgie
04 Faculty of Medicine > Service Sector > Institute of Pathology
04 Faculty of Medicine > Department of Orthopaedic, Plastic and Hand Surgery (DOPH) > Clinic of Plastic and Hand Surgery
04 Faculty of Medicine > Department of Radiology, Neuroradiology and Nuclear Medicine (DRNN) > Institute of Diagnostic and Interventional Neuroradiology

UniBE Contributor:

Vajtai, Istvan; Vögelin, Esther; Ozdoba, Christoph and Hewer, Ekkehard

Subjects:

500 Science > 570 Life sciences; biology
600 Technology > 610 Medicine & health

ISSN:

0148-639X

Publisher:

John Wiley & Sons

Language:

English

Submitter:

Martin Zbinden

Date Deposited:

14 Oct 2014 08:17

Last Modified:

29 Oct 2015 11:44

Publisher DOI:

10.1002/mus.24165

PubMed ID:

24395394

Web of Science ID:

000335954400019

Uncontrolled Keywords:

EWSR1/WT1 fusion nerve biopsy, brachial plexopathy, desmoplastic small round cell tumor, positron emission tomography

BORIS DOI:

10.7892/boris.48090

URI:

https://boris.unibe.ch/id/eprint/48090

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