Cervical Nerve Root Synovial Sarcoma in a Child with Chromosomal (X;18) Translocation

Abstract

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.

An infant with intradural extramedullary synovial sarcoma: the youngest case in the literature

“…To date, there are only two pediatric reports originating from the intradural extramedullary area. 7,8 These patients' characteristics are summarized in Table I. Our patient is the third and the youngest among all.…”

“…Most of the reported cases had local or distant metastases and they were lost due to disease progression despite treatment. 6,7 Two reported pediatric cases in the literature were also lost because of disease progression even though they were operated on and later treated with chemotherapy and radiotherapy.…”

“…To our knowledge, this is the youngest case of intradural extramedullary spinal SS in the literature. 7,8 Case Report A 14-month-old girl presented with difficulty in walking ongoing for a week. Trauma or other predisposing factors were not identified.…”

Pediatric primitive intraneural synovial sarcoma of L-5 nerve root

“…To date, only 24 cases have been reported in the literature, 9 of which occurred in children and adolescents (Table 1). 3,4,6,7,14,18,19,23,26,[28][29][30]34,35 The radiological pattern is nonspecific: 30% of cases are characterized by calcifications seen on radiographs or CT scans, suggesting a benign lesion. 2 Synovial sarcomas may have variable intensity and enhancement on MRI; they are relatively isointense or hypointense to muscle and isointense or slightly hyperintense to fat and tend to be oval, sharply marginated tumors.…”

Primary pediatric intraspinal sarcomas

“…72 Only a single case of intramedullary EES has been reported previously. 82 Several cases of intradural sarcomas associated with spinal nerve roots, including synovial sarcoma 21,32 and clear cell sarcoma, 70 have been reported. Primitive neuroectodermal tumors, which are part of the ESFTs, have also been described arising from a spinal nerve root.…”