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: lvalentini@istituto-besta.it.

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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Figures

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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.

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