Synovial Sarcoma of Peripheral Nerves

John S.A. Chrisinger, Usama Salem, Lars Gunnar Kindblom, Behrang Amini, Magnus Hansson, Jeanne M. Meis

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

Synovial sarcoma of peripheral nerve (SSPN) is rare with only 26 cases reported in English. SSPN is often mistaken for a benign or malignant peripheral nerve sheath tumor (PNST) by clinicians and pathologists. Fifteen cases of SSPN were retrieved from the pathology files of 3 institutions. All tumors arose in a nerve based on imaging and/or operative findings and the diagnoses were histologically confirmed. Neuropathic symptoms predominated in 11 women and 4 men, 19- to 62-year-old (median, 39 years) with tumors involving the ulnar (5), median (3), peroneal (3) or sciatic (2) nerve, or the L4 or T1 nerve root (2). Tumor sizes ranged from 2 to 13 cm (median, 3.8 cm). The leading clinical diagnosis was PNST (9). Treatment was surgical (14) supplemented with radiation therapy (8) and chemotherapy (6). Fourteen tumors were monophasic and 1 was biphasic; 4 had poorly differentiated (PD) foci (1 rhabdoid). Diagnoses in 12 cases were verified by fluorescence in situ hybridization, reverse transcription polymerase chain reaction or both methods. Follow-up in 14 patients (median, 32 mo) revealed that 2/4 patients with PD tumors died with pulmonary metastases; another was alive with no current evidence of disease (NED) following 2 local recurrences, while the fourth had NED. In contrast, 9/10 patients without PD tumors were alive (7 NED) and 1 died at 12 months with pulmonary infiltrates. SSPN is under-recognized clinically and histologically as it mimics benign and malignant PNST. Molecular analysis is recommended to confirm the diagnosis. PD foci, including rhabdoid areas, may portend a worse outcome, similar to non-neural-based tumors.

Original languageEnglish (US)
Pages (from-to)1087-1096
Number of pages10
JournalAmerican Journal of Surgical Pathology
Volume41
Issue number8
DOIs
StatePublished - Jan 1 2017
Externally publishedYes

Fingerprint

Synovial Sarcoma
Peripheral Nerves
Neoplasms
Neurilemmoma
Nerve Sheath Neoplasms
Lung
Sciatic Nerve
Fluorescence In Situ Hybridization
Reverse Transcription
Radiotherapy
Pathology
Neoplasm Metastasis
Recurrence
Drug Therapy
Polymerase Chain Reaction

Keywords

  • intraneural
  • peripheral nerve sheath tumor
  • synovial sarcoma

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine

Cite this

Chrisinger, J. S. A., Salem, U., Kindblom, L. G., Amini, B., Hansson, M., & Meis, J. M. (2017). Synovial Sarcoma of Peripheral Nerves. American Journal of Surgical Pathology, 41(8), 1087-1096. https://doi.org/10.1097/PAS.0000000000000874

Synovial Sarcoma of Peripheral Nerves. / Chrisinger, John S.A.; Salem, Usama; Kindblom, Lars Gunnar; Amini, Behrang; Hansson, Magnus; Meis, Jeanne M.

In: American Journal of Surgical Pathology, Vol. 41, No. 8, 01.01.2017, p. 1087-1096.

Research output: Contribution to journalArticle

Chrisinger, JSA, Salem, U, Kindblom, LG, Amini, B, Hansson, M & Meis, JM 2017, 'Synovial Sarcoma of Peripheral Nerves', American Journal of Surgical Pathology, vol. 41, no. 8, pp. 1087-1096. https://doi.org/10.1097/PAS.0000000000000874
Chrisinger JSA, Salem U, Kindblom LG, Amini B, Hansson M, Meis JM. Synovial Sarcoma of Peripheral Nerves. American Journal of Surgical Pathology. 2017 Jan 1;41(8):1087-1096. https://doi.org/10.1097/PAS.0000000000000874
Chrisinger, John S.A. ; Salem, Usama ; Kindblom, Lars Gunnar ; Amini, Behrang ; Hansson, Magnus ; Meis, Jeanne M. / Synovial Sarcoma of Peripheral Nerves. In: American Journal of Surgical Pathology. 2017 ; Vol. 41, No. 8. pp. 1087-1096.
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