Synovial sarcoma of the cauda equina

Case report

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Primary synovial sarcoma originating from the cauda equina is extremely rare. Only one case, involving an 11-year-old girl, has been reported. The authors describe the case of a 23-year-old woman with a primary synovial sarcoma of the cauda equina.

The patient visited a local hospital and described a 2-month history of low-back pain. She was referred to the authors' hospital for further evaluation. On physical examination, she had a straight–leg raising result of 70° bilaterally. Motor examination revealed Grade 4/5 strength in the bilateral extensor hallux longus muscles. There was normal sensation to light touch and vibration in the lower extremities. Sagittal Gd-enhanced T1-weighted MR imaging demonstrated an intradural, extramedullary, and uniformly enhancing mass that extended from L-3 to L-4. The mass was totally resected and adjuvant local radiation therapy was administered. Reverse transcriptase polymerase chain reaction (RT-PCR) of a paraffin-embedded tissue sample revealed SYT-SSX fusion transcripts, and the diagnosis of synovial sarcoma was confirmed. Five and a half years after surgery, the patient is free of local recurrence and metastatic disease. The RT-PCR detection of SYT-SSX fusion transcripts played a key role in establishing the diagnosis of synovial sarcoma of the cauda equina. Complete resection of the mass with adjuvant local radiation therapy proved to be effective.

Abbreviations used in this paper: MPNST = malignant peripheral nerve sheath tumor; RT-PCR = reverse transcriptase polymerase chain reaction.

Article Information

Address correspondence to: Ikuho Yonezawa, M.D., Department of Orthopaedic Surgery, Juntendo University, 2-1-1 Hongo, Bunkyo-ku, Tokyo 113-8421, Japan. email: yoza@juntendo.ac.jp.

Please include this information when citing this paper: published online November 18, 2011; DOI: 10.3171/2011.10.SPINE11359.

© AANS, except where prohibited by US copyright law.

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Figures

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    Preoperative sagittal Gd-enhanced T1-weighted MR image demonstrating an intradural, extramedullary, and uniformly enhancing mass extending from L-3 to L-4.

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    Photomicrographs. A: Spindle-shaped cells proliferating in a fascicular fashion with stromal hyalinization. H & E, original magnification × 100. B: Pseudorosette formations are focally seen. H & E, original magnification × 100. C: Pseudopapillary structures are frequently seen. H & E, original magnification × 40. D: Distinct expression of S100 protein as seen in MPNSTs is not demonstrated. Original magnification × 100.

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    Upper: A PCR product for SYT-SSX chimeric fusion transcript was detected. contl. = control. Lower: Direct sequencing confirmed that this PCR product contained a sequence identical to that of SYT-SSX1. SYT-SSX1 and SYT-SSX2 sequences are of this amplified region. Characters shown in red indicate different bases between sequences.

References

1

Cadman NLSoule EHKelly PJ: Synvial sarcoma; an analysis of 34 tumors. Cancer 18:6136271965

2

Canter RJQin LXMaki RGBrennan MFLadanyi MSinger S: A synovial sarcoma-specific preoperative nomogram supports a survival benefit to ifosfamide-based chemotherapy and improves risk stratification for patients. Clin Cancer Res 14:819181972008

3

Eilber FCBrennan MFEilber FREckardt JJGrobmyer SRRiedel E: Chemotherapy is associated with improved survival in adult patients with primary extremity synovial sarcoma. Ann Surg 246:1051132007

4

Ferrari AGronchi ACasanova MMeazza CGandola LCollini P: Synovial sarcoma: a retrospective analysis of 271 patients of all ages treated at a single institution. Cancer 101:6276342004

5

Greene SHawkins DSRutledge JCTsuchiya KDDouglas JEllenbogen RG: Pediatric intradural extramedullary synovial sarcoma: case report. Neurosurgery 59:E13392006

6

Hasegawa TYokoyama RMatsuno YShimoda THirohashi S: Prognostic significance of histologic grade and nuclear expression of beta-catenin in synovial sarcoma. Hum Pathol 32:2572632001

7

Italiano APenel NRobin YMBui BLe Cesne APiperno-Neumann S: Neo/adjuvant chemotherapy does not improve outcome in resected primary synovial sarcoma: a study of the French Sarcoma Group. Ann Oncol 20:4254302009

8

Karn CMSocinski MAFletcher JACorson JMCraighead JE: Cardiac synovial sarcoma with translocation (X;18) associated with asbestos exposure. Cancer 73:74781994

9

Limon JMrozek KMandahl NNedoszytko BVerhest ARys J: Cytogenetics of synovial sarcoma: presentation of ten new cases and review of the literature. Genes Chromosomes Cancer 3:3383451991

10

Nagayama SKatagiri TTsunoda THosaka TNakashima YAraki N: Genome-wide analysis of gene expression in synovial sarcomas using a cDNA microarray. Cancer Res 62:585958662002

11

Naka NTakenaka SAraki NMiwa THashimoto NYoshioka K: Synovial sarcoma is a stem cell malignancy. Stem Cells 28:111911312010

12

Parfitt JRXu JKontozoglou TOluwafemi ARDriman DK: Primary monophasic synovial sarcoma of the colon. Histopathology 50:5215232007

13

Rekhi BIngle AKumar RDeSouza MADikshit RJambhekar NA: Malignant peripheral nerve sheath tumors: clinicopathological profile of 63 cases diagnosed at a tertiary cancer referral center in Mumbai, India. Indian J Pathol Microbiol 53:6116182010

14

Schmidt DThum PHarms DTreuner J: Synovial sarcoma in children and adolescents. A report from the Kiel Pediatric Tumor Registry. Cancer 67:166716721991

15

Singer SBaldini EHDemetri GDFletcher JACorson JM: Synovial sarcoma: prognostic significance of tumor size, margin of resection, and mitotic activity for survival. J Clin Oncol 14:120112081996

16

Spillane AJA'Hern RJudson IRFisher CThomas JM: Synovial sarcoma: a clinicopathologic, staging, and prognostic assessment. J Clin Oncol 18:379438032000

17

Srivastava ANielsen PGDal Cin PRosenberg AE: Monophasic synovial sarcoma of the liver. Arch Pathol Lab Med 129:104710492005

18

ten Heuvel SEHoekstra HJBastiaannet ESuurmeijer AJ: The classic prognostic factors tumor stage, tumor size, and tumor grade are the strongest predictors of outcome in synovial sarcoma: no role for SSX fusion type or ezrin expression. Appl Immunohistochem Mol Morphol 17:1891952009

19

Trassard MLe Doussal VHacène KTerrier PRanchère DGuillou L: Prognostic factors in localized primary synovial sarcoma: a multicenter study of 128 adult patients. J Clin Oncol 19:5255342001

20

Tsuji SHisaoka MMorimitsu YHashimoto HShimajiri SKomiya S: Detection of SYT-SSX fusion transcripts in synovial sarcoma by reverse transcription-polymerase chain reaction using archival paraffin-embedded tissues. Am J Pathol 153:180718121998

21

Turc-Carel CDal Cin PLimon JRao ULi FPCorson JM: Involvement of chromosome X in primary cytogenetic change in human neoplasia: nonrandom translocation in synovial sarcoma. Proc Natl Acad Sci U S A 84:198119851987

22

Weiss SWGoldblum JR: Enzinger and Weiss's Soft Tissue Tumors ed 4PhiladelphiaMosby2001

23

Zenmyo MKomiya SHamada THiraoka KNagata KTsuji S: Intraneural monophasic synovial sarcoma: a case report. Spine (Phila Pa 1976) 26:3103132001

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