Cervical nerve root synovial sarcoma in a child with chromosomal (X;18) translocation. Case report and review of the literature.
We report on an 11-year-old female with a history of cervicobrachialgia and progressive weakness of the right arm. Cervical spine MRI showed an enhancing heterogeneous intradural mass occupying the right C6-C7 foramen. She underwent a right C6-C7 foraminotomy with a complete macroscopic removal of the lesion. Pathological examination revealed a synovial sarcoma. Treatment was completed by chemotherapy and proton radiotherapy, and the girl remained free of symptoms for 3 years. After appearance of new symptoms, a local recurrence was confirmed, and despite aggressive treatment with salvage chemotherapy and radiotherapy, the disease progressed beyond medical control, and the child died, 6 years after diagnosis. Early recognition of this rare entity compared to its more benign differential diagnosis is crucial, as an aggressive management is needed.
de Ribaupierre
Sandrine
S
Service of Neurosurgery, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland. sandrine.de-ribaupierre@chuv.ch
Vernet
Olivier
O
Beck-Popovic
Maya
M
Meagher-Villemure
Kathleen
K
Rilliet
Bénédict
B
D002574:Cervical Vertebrae / Q000473:pathology* / Q000601:surgery; D002648:Child; D002887:Chromosomes, Human, Pair 18 / Q000235:genetics*; D041321:Chromosomes, Human, X / Q000235:genetics*; D005260:Female; D006801:Humans; D010524:Peripheral Nervous System Neoplasms / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery; D013584:Sarcoma, Synovial / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery; D013126:Spinal Nerve Roots / Q000473:pathology* / Q000601:surgery; D014178:Translocation, Genetic / Q000235:genetics*