Pediatric primitive intraneural synovial sarcoma of L-5 nerve root

Case report

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Primitive intraneural synovial sarcomas are rare in children. The authors report the case of a 7-year-old girl affected by intraneural synovial sarcoma of a lumbar nerve root, the first such lesion in this location described in a child. The lesion mimicked a schwannoma clinically and radiologically. There was long-lasting leg pain in a radicular distribution, and a well-demarcated intraneural tumor was seen on MRI. On this basis, the first resection was conservative. However, histological examination documented a classic biphasic synovial sarcoma, which was confirmed by immunohistochemistry. After radical resection and adjuvant treatment, complete disease control was achieved and verified at 5-year follow-up. This case strongly suggests that early diagnosis and a multidisciplinary approach to this unusual spinal lesion are essential to achieving a better prognosis.

Abbreviations used in this paper:FISH = fluorescence in situ hybridization; IRS = Intergroup Rhabdomyosarcoma Study; MPNST = malignant peripheral nerve sheath tumor; RT-PCR = reverse transcription polymerase chain reaction.

Article Information

Address correspondence to: Laura G. Valentini, M.D., Department of Neurosurgery, Fondazione IRCCS Istituto Neurologico Carlo Besta, Via Celoria 11, 20133 Milano, Italy. email:

Please include this information when citing this paper: published online February 15, 2013; DOI: 10.3171/2013.1.PEDS12181.

© AANS, except where prohibited by US copyright law.



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    A and B: Contrast-enhanced T1-weighted sagittal MR images of spine show a well-defined enhancing mass (arrows) entirely occupying the left foramen of L-5. C and D: T1-weighted axial MR images show an hourglass-shaped mass (asterisk) extending inside and outside the vertebral canal (C) that homogeneously enhances after contrast injection (D). The left conjugate foramen is enlarged.

  • View in gallery

    Photomicrograph demonstrating the typical picture of a biphasic synovial sarcoma, with both epithelial and spindle cell components. H & E, original magnification ×100.



Brecht IBFerrari AInt-Veen CSchuck AMattke ACCasanova M: Grossly-resected synovial sarcoma treated by the German and Italian Pediatric Soft Tissue Sarcoma Cooperative Groups: discussion on the role of adjuvant therapies. Pediatr Blood Cancer 46:11172006


Cadman NLSoule EHKelly PJ: Synovial sarcoma: an analysis of 134 tumors. Cancer 18:6136271965


Chesser TJSGeraghty JMClarke AM: Intraneural synovial sarcoma of the median nerve. J Hand Surg Br 24:3733751999


Chu PGBenhattar JWeiss LMMeagher-Villemure K: Intraneural synovial sarcoma: two cases. Mod Pathol 17:2582632004


Coindre JMHostein IBenhattar JLussan CRivel JGuillou L: Malignant peripheral nerve sheath tumors are t(X;18)-negative sarcomas. Molecular analysis of 25 cases occurring in neurofibromatosis type 1 patients, using two different RTPCR-based methods of detection. Mod Pathol 15:5895922002


Cugola LPisa R: Synovial sarcoma: with radial nerve involvement. J Hand Surg Br 10:2432441985


de Ribaupierre SVernet OBeck-Popovic MMeagher-Villemure KRilliet B: Cervical nerve root synovial sarcoma in a child with chromosomal (X;18) translocation. Case report and review of the literature. Pediatr Neurosurg 43:3823852007


Ferrari ABisogno GAlaggio RCecchetto GCollini PRosolen A: Synovial sarcoma of children and adolescents: the prognostic role of axial sites. Eur J Cancer 44:120212092008


Ferrari ACasanova M: New concepts for the treatment of pediatric nonrhabdomyosarcoma soft tissue sarcomas. Expert Rev Anticancer Ther 5:3073182005


Ferrari ACasanova MMassimino MLuksch RCefalo GLombardi F: Synovial sarcoma: report of a series of 25 consecutive children from a single institution. Med Pediatr Oncol 32:32371999


Fletcher CDMUnni KKMertens F: World Health Organisation Classification of Tumours: Pathology and Genetics of Tumours of Soft Tissue and Bone LyonIARC Press2002


Foo WCCruise MWWick MRHornick JL: Immunohistochemical staining for TLE1 distinguishes synovial sarcoma from histologic mimics. Am J Clin Pathol 135:8398442011


Golouh RVuzevski VBracko Mvan der Heul ROCervek J: Synovial sarcoma: a clinicopathological study of 36 cases. J Surg Oncol 45:20281990


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


Knösel THeretsch SAltendorf-Hofmann ARichter PKatenkamp KKatenkamp D: TLE1 is a robust diagnostic biomarker for synovial sarcomas and correlates with t(X;18): analysis of 319 cases. Eur J Cancer 46:117011762010


Kosemehmetoglu KVrana JAFolpe AL: TLE1 expression is not specific for synovial sarcoma: a whole section study of 163 soft tissue and bone neoplasms. Mod Pathol 22:8728782009


Ladenstein RTreuner JKoscielniak Ed'Oleire FKeim MGadner H: Synovial sarcoma of childhood and adolescence. Report of the German CWS-81 study. Cancer 71:364736551993


Lestou VSO'Connell JXRobichaud MSalski CMathers JMaguire J: Cryptic t(X;18), ins(6;18), and SYT-SSX2 gene fusion in a case of intraneural monophasic synovial sarcoma. Cancer Genet Cytogenet 138:1531562002


Lipira ABKasukurthi RRay WZPruzansky MEMackinnon SE: Intraneural synovial sarcoma of the median nerve. Rare Tumors 2:e322010


Maurer HMBeltangady MGehan EACrist WHammond DHays DM: The Intergroup Rhabdomyosarcoma Study-I. A final report. Cancer 61:2092201988


McCarville MBSpunt SLSkapek SXPappo AS: Synovial sarcoma in pediatric patients. AJR Am J Roentgenol 179:7978012002


Mullen JRZagars GK: Synovial sarcoma outcome following conservation surgery and radiotherapy. Radiother Oncol 33:23301994


O'Connell JXBrowne WLGropper PTBerean KW: Intraneural biphasic synovial sarcoma: an alternative “glandular” tumor of peripheral nerve. Mod Pathol 9:7387411996


Okcu MFMunsell MTreuner JMattke APappo ACain A: Synovial sarcoma of childhood and adolescence: a multicenter, multivariate analysis of outcome. J Clin Oncol 21:160216112003


Pappo ASFontanesi JLuo XRao BNParham DMHurwitz C: Synovial sarcoma in children and adolescents: the St Jude Children's Research Hospital experience. J Clin Oncol 12:236023661994


Rinehart GCMustoe TAWeeks PM: Management of synovial sarcoma of the median nerve at the elbow. Plast Reconstr Surg 83:5285321989


Sandberg AABridge JA: Updates on the cytogenetics and molecular genetics of bone and soft tissue tumors. Synovial sarcoma. Cancer Genet Cytogenet 133:1232002


Scheithauer BWAmrami KKFolpe ALSilva AIEdgar MAWoodruff JM: Synovial sarcoma of nerve. Hum Pathol 42:5685772011


Spielmann AJanzen DLO'Connell JXMunk PL: Intraneural synovial sarcoma. Skeletal Radiol 26:6776811997


Tacconi LThom MThomas DG: Primary monophasic synovial sarcoma of the brachial plexus: report of a case and review of the literature. Clin Neurol Neurosurg 98:2492521996


Tamborini EAgus VPerrone FPapini DRomanò RPasini B: Lack of SYT-SSX fusion transcripts in malignant peripheral nerve sheath tumors on RT-PCR analysis of 34 archival cases. Lab Invest 82:6096182002


Trojani MContesso GCoindre JMRouesse JBui NBde Mascarel A: Soft-tissue sarcomas of adults; study of pathological prognostic variables and definition of a histopathological grading system. Int J Cancer 33:37421984


Uehara HYamasaki KFukushima TYamashita AMarutsuka KNakano S: Intraneural synovial sarcoma originating from the median nerve. Neurol Med Chir (Tokyo) 48:77822008


Weinreb IPerez-Ordoñez BGuha AKiehl TR: Mucinous, gland predominant synovial sarcoma of a large peripheral nerve: a rare case closely mimicking metastatic mucinous carcinoma. J Clin Pathol 61:6726762008


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




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