Synovial sarcoma of nerve

Bernd W. Scheithauer, Kimberly K. Amrami, Andrew L. Folpe, Ana I. Silva, Mark A. Edgar, James M. Woodruff, Allan D Levi, Robert J. Spinner

Research output: Contribution to journalArticle

20 Citations (Scopus)

Abstract

Tumors of peripheral nerve are largely neuroectodermal in nature and derived from 2 elements of nerve, Schwann or perineurial cells. In contrast, mesenchymal tumors affecting peripheral nerve are rare and are derived mainly from epineurial connective tissue. The spectrum of the latter is broad and includes lipoma, vascular neoplasms, hematopoietic tumors, and even meningioma. Of malignant peripheral nerve neoplasms, the vast majority are primary peripheral nerve sheath tumors. Malignancies of mesenchymal type are much less common. To date, only 12 cases of synovial sarcoma of nerve have been described. Whereas in the past, parallels were drawn between synovial sarcoma and malignant glandular schwannoma, an uncommon form of malignant peripheral nerve sheath tumor, molecular genetics have since clarified the distinction. Herein, we report 10 additional examples of molecularly confirmed synovial sarcoma, all arising within minor or major nerves. Affecting 7 female and 3 male patients, 4 tumors occurred in pediatric patients. Clinically and radiologically, most lesions were initially thought to be benign nerve sheath tumors. On reinterpretation of imaging, they were considered indeterminate in nature with some features suspicious for malignancy. Synovial sarcoma of nerve, albeit rare, seems to behave in a manner similar to its more common, soft tissue counterpart. Those affecting nerve have a variable prognosis. Definitive recommendations regarding surgery and adjuvant therapies await additional reports and long-term follow-up. The literature is reviewed and a meta-analysis is performed with respect to clinicopathologic features versus outcome.

Original languageEnglish
Pages (from-to)568-577
Number of pages10
JournalHuman Pathology
Volume42
Issue number4
DOIs
StatePublished - Apr 1 2011

Fingerprint

Synovial Sarcoma
Peripheral Nervous System Neoplasms
Nerve Sheath Neoplasms
Neurilemmoma
Neoplasms
Vascular Neoplasms
Lipoma
Meningioma
Connective Tissue
Meta-Analysis
Molecular Biology
Pediatrics

Keywords

  • Immunohistochemistry
  • Nerve
  • Sarcoma
  • Synovial sarcoma

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

Scheithauer, B. W., Amrami, K. K., Folpe, A. L., Silva, A. I., Edgar, M. A., Woodruff, J. M., ... Spinner, R. J. (2011). Synovial sarcoma of nerve. Human Pathology, 42(4), 568-577. https://doi.org/10.1016/j.humpath.2010.08.019

Synovial sarcoma of nerve. / Scheithauer, Bernd W.; Amrami, Kimberly K.; Folpe, Andrew L.; Silva, Ana I.; Edgar, Mark A.; Woodruff, James M.; Levi, Allan D; Spinner, Robert J.

In: Human Pathology, Vol. 42, No. 4, 01.04.2011, p. 568-577.

Research output: Contribution to journalArticle

Scheithauer, BW, Amrami, KK, Folpe, AL, Silva, AI, Edgar, MA, Woodruff, JM, Levi, AD & Spinner, RJ 2011, 'Synovial sarcoma of nerve', Human Pathology, vol. 42, no. 4, pp. 568-577. https://doi.org/10.1016/j.humpath.2010.08.019
Scheithauer BW, Amrami KK, Folpe AL, Silva AI, Edgar MA, Woodruff JM et al. Synovial sarcoma of nerve. Human Pathology. 2011 Apr 1;42(4):568-577. https://doi.org/10.1016/j.humpath.2010.08.019
Scheithauer, Bernd W. ; Amrami, Kimberly K. ; Folpe, Andrew L. ; Silva, Ana I. ; Edgar, Mark A. ; Woodruff, James M. ; Levi, Allan D ; Spinner, Robert J. / Synovial sarcoma of nerve. In: Human Pathology. 2011 ; Vol. 42, No. 4. pp. 568-577.
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