Key result
Maximal treatment fails to prevent rapid leptomeningeal metastasis and death in pediatric intradural synovial sarcoma.
Case Report (n=1)
Intradural synovial sarcoma is a highly aggressive tumor characterized by rapid progression of leptomeningeal metastasis despite maximal surgical and chemoradiation therapy.
Indicates dismal prognosis for pediatric intradural synovial sarcoma despite maximal therapy; leaves optimal management open for prospective study.
OBJECTIVE: The diagnosis of intradural synovial sarcoma has not been previously published. This report provides a summary of the literature on this tumor and on tumors arising in this location, as well as a description of this patient's clinical course. CLINICAL PRESENTATION: An 11-year-old girl presented with back pain and radiculopathy. A magnetic resonance imaging scan of the spine revealed a spinal intradural, extramedullary mass at L2-L4 and four additional nodules of enhancement. INTERVENTION: The mass was nearly totally resected. Radiation and chemotherapy were administered. Intracranial metastases became evident during treatment. The patient died of the disease 14 months after diagnosis. CONCLUSION: The rapid progression of leptomeningeal metastasis despite maximal treatment demonstrates the aggressive nature of the tumor and the need for further study.
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Greene et al. (2006) conducted a case report in Intradural synovial sarcoma (n=1). Resection, radiation, and chemotherapy was evaluated on Clinical course and survival. An 11-year-old girl with intradural synovial sarcoma experienced rapid progression of leptomeningeal metastasis and died 14 months after diagnosis despite maximal treatment.
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