RESECTION OF PRIMARY TUMOR AT DIAGNOSIS IN STAGE IV-S NEUROBLASTOMA -DOES IT AFFECT THE CLINICAL COURSE

Purpose: To determine whether resection of primary tumor has a favorab le influence on outcome of infants (age 0 to 11 months) with stage IV- S neuroblastoma.Patients and Methods: Between March 1976 and December 1993, 97 infants with previously untreated neuroblastoma diagnosed in 21 Italian institutions were classified as having stage IV-S disease. Seventy percent were younger than 4 months. Adrenal was the primary tu mor site in 64 of 85 patients with a recognizable primary tumor. Liver was the organ most often infiltrated by the tumor (82 patients), foll owed by bone marrow and skin. Results: The overall survival (OS) rate at 5 years is 80% and event-free survival (EFS) rate 68%. In 24 infant s, the effect of resection of primary tumor could not be evaluated bec ause of rapidly fatal disease progression (n = 8), absence of a primar y tumor (n = 12), or partial resection (n = 4). Of 73 assessable patie nts, 26 underwent primary tumor resection at diagnosis: one died of su rgical complications, one relapsed locally and died, and two others re lapsed (one of these two locally) and survived, for a 5-year OS rate o f 92% and EFS rate of 84%. Of the remaining 47 patients who did not un dergo primary tumor resection at diagnosis 11 suffered unfavorable eve nts, of whom five died, for an OS rate of 89% and EFS rate of 75% (no significant difference from previous group). Disease recurred at the p rimary tumor site in only one of five who died, and in only one of six survivors of progression or relapse; in these patients, the primary t umor, located in the mediastinum, was successfully resected. Conclusio n: infants who underwent resection of the primary tumor at diagnosis h ad no better Outcome than those in whom the decision wets made not to operate. (C) 1996 by American Society of Clinical Oncology.