Pediatric primitive intraneural synovial sarcoma of L-5 nerve root

Case report

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  • 1 Department of Neurosurgery and
  • | 5 Neuroradiology Service, Fondazione IRCCS Istituto Neurologico Carlo Besta;
  • | 3 Department of Diagnostic Pathology and Laboratory Medicine and
  • | 4 Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy; and
  • | 2 Institute of Neuropathology, DGNN Brain Tumor Reference Center, University of Bonn Medical Center, Bonn, Germany
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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.

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