Bone marrow metastases at diagnosis in children and adolescents with rhabdomyosarcoma. A report from the intergroup rhabdomyosarcoma study.
Thirty children and adolescents with rhabdomyosarcoma (RMS) entered on IRS-1 in a 5-year period had bone marrow metastases (BMM) at diagnosis. BMM at diagnosis occurred in 30/103 patients (29%) with Clinical Group IV RMS. On the average, patients with BMM were older with more extremity primaries than other IRS-1 patients. Alveolar histology was present in 15/30 patients (50%) with BMM, compared to 19% on IRS-1. Eleven patients received vincristine, dactinomycin, cyclophosphamide (VAC), while 19 patients received VAC with Adriamycin (ADR). The overall complete response (CR) rate was 60%. The median disease-free survival times by treatment were 17 weeks with VAC and 32 weeks for VAC + ADR (P greater than 0.10). Relapse from CR in Clinical Group IV patients with BMM occurred in 16/18 patients (89%) and was significantly higher than in 17/29 Clinical Group IV patients (59%) without BMM (P less than 0.035). BMM at diagnosis in children and adolescents with RMS is frequently associated with alveolar histology and is a poor prognostic sign.
D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001855:Bone Marrow Diseases / Q000188:drug therapy*; D001859:Bone Neoplasms / Q000188:drug therapy*; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000008:administration & dosage; D003609:Dactinomycin / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D005121:Extremities; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D011379:Prognosis; D012042:Registries; D012208:Rhabdomyosarcoma / Q000188:drug therapy* / Q000473:pathology; D013997:Time Factors; D014750:Vincristine / Q000008:administration & dosage
D003609:Dactinomycin; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide