Journal of Clinical Oncology, Vol 14, 1537-1544, Copyright © 1996 by American Society of Clinical Oncology
Resection of primary tumor at diagnosis in stage IV-S neuroblastoma: does it affect the clinical course?
M Guglielmi, B De Bernardi, A Rizzo, S Federici, C Boglino, F Siracusa, A Leggio, F Cozzi, G Cecchetto, L Musi, T Bardini, AM Fagnani, GC Bartoli, A Pampaloni, D Rogers, M Conte, C Milanaccio and P Bruzzi
Department of Hematology-Oncology, Giannina Gaslini Children's Hospital, Genova, Italy.
PURPOSE: To determine whether resection of primary tumor has a favorable
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 tumor site in 64 of 85
patients with a recognizable primary tumor. Liver was the organ most often
infiltrated by the tumor (82 patients), followed by bone marrow and skin.
RESULTS: The overall survival (OS) rate at 5 years in 80% and event-free
survival (EFS) rate 68%. In 24 infants, the effect of resection of primary
tumor could not be evaluated because of rapidly fatal disease progression
(n = 8), absence of a primary tumor (n = 12), or partial resection (n = 4).
Of 73 assessable patients, 26 underwent primary tumor resection at
diagnosis: one died of surgical complications, one relapsed locally and
died, and two others relapsed (one of these two locally) and survived, for
a 5-year OS rate of 92% and EFS rate of 84%. Of the remaining 47 patients
who did not undergo primary tumor resection at diagnosis 11 suffered
unfavorable events, 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 primary tumor site in only one five who died, and in only one of six
survivors of progression or relapse; in these patients, the primary tumor,
located in the mediastinum, was successfully resected. CONCLUSION: Infants
who underwent resection of the primary tumor at diagnosis had no better
outcome than those in whom the decision was made not to operate.

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