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[Dumbbell neuroblastoma. Experience at the Gustave Roussy Institute in 38 cases treated from 1982 to 1987].

作者信息

Plantaz D, Hartmann O, Kalifa C, Sainte-Rose C, Passagia J G, Lemerle J

机构信息

Service de Pédiatrie, Institut Gustave-Roussy, CHU de Grenoble.

出版信息

Arch Fr Pediatr. 1991 Oct;48(8):529-33.

PMID:1768192
Abstract

Among the 282 neuroblastomas treated at the Institut Gustave-Roussy between 1982 and 1987, 38 dumbbell forms were observed. Therapeutic approaches included: 1) An initial laminectomy in forms with neurological deficit; 2) Surgical excision of the primary tumor; 3) Preoperative chemotherapy for metastatic forms and non-metastatic forms in which primary tumors considered unresectable at diagnosis; 4) Radiation therapy on macroscopic residual disease. Twenty three of 38 children presented with a neurological deficit. A laminectomy was performed in 21 cases. Neurological recovery was good in 8 cases, partial in 5 cases and absent in 5 cases. Three patients were aggravated after the procedure. The event free survival was 76%. This high survival rate is linked with: 1) The predominantly non metastatic stages (25/38); 2) A high proportion of children under 1 year of age (25/38); 3) A high proportion of thoracic locations. Out of the 29 survivors, there were 10 cases of major neurological sequelae (34%) and 9 cases of major orthopedic sequelae (31%). The coexistence of a serious functional prognosis and an excellent vital prognosis led us to analyse the therapeutic modalities and reevaluate the necessity of routine initial neurosurgical excision by laminectomy, and using a first line chemotherapy in selected indications.

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