PRIMARY INTRASPINAL SOFT-TISSUE SARCOMA IN CHILDHOOD - REPORT OF 2 CASES WITH A REVIEW OF THE LITERATURE

Two young children who presented with lower spinal cord dysfunction ma nifested by bilateral leg weakness and urinary retention were diagnose d with intraspinal soft-tissue sarcoma. Neither patient had a signific ant extradural mass. Both tumors had histochemical features of rhabdom yosarcoma. Temporary responses were noted after combination chemothera py either with vincristine, actinomycin D, and cyclophosphamide or wit h ifosfamide/mesna and etoposide. However, both patients developed unc ontrollable cerebrospinal fluid (CSF) dissemination of tumor and died within 6 months of diagnosis, despite intrathecal chemotherapy and irr adiation for one and very high-dose intravenous methotrexate (33 g/m2) for the other. This rare tumor can respond to parenteral antisarcoma chemotherapy, but better strategies are needed to prevent CSF spread a nd ultimate demise. Early institution of intrathecal cytostatic agents may retard or prevent CSF dissemination and prolong survival. (C) 199 4 Wiley-Liss, Inc.