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Resection of primary tumor at diagnosis in stage IV-S neuroblastoma: does it affect the clinical course?
M Guglielmi1, B De Bernardi, A Rizzo
1Department of Hematology-Oncology, Giannina Gaslini Children's Hospital, Genova, Italy.
Insights
Resecting the primary tumor in infants with stage IV-S neuroblastoma did not improve outcomes. Survival rates were similar whether or not surgery was performed at diagnosis.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Neuroblastoma Research
Background:
- Stage IV-S neuroblastoma is a specific form of the disease in infants.
- The role of primary tumor resection in this population has been debated.
- Understanding optimal treatment strategies is crucial for improving infant outcomes.
Purpose of the Study:
- To evaluate the impact of primary tumor resection on the survival of infants with stage IV-S neuroblastoma.
- To compare outcomes between infants who underwent primary tumor resection and those who did not.
Main Methods:
- A cohort of 97 infants with stage IV-S neuroblastoma was studied across 21 Italian institutions.
- Data were collected on primary tumor site, metastatic involvement (liver, bone marrow, skin), and surgical intervention.
- Survival rates, including overall survival (OS) and event-free survival (EFS), were analyzed.
Main Results:
- The overall cohort showed a 5-year OS rate of 80% and EFS rate of 68%.
- Among 73 assessable patients, those who underwent primary tumor resection (n=26) had a 5-year OS of 92% and EFS of 84%.
- Patients not undergoing resection (n=47) had similar outcomes with a 5-year OS of 89% and EFS of 75%, indicating no significant difference.
Conclusions:
- Primary tumor resection at diagnosis in infants with stage IV-S neuroblastoma does not appear to confer a survival advantage.
- The decision not to operate on the primary tumor did not negatively impact outcomes compared to resection.
- Further research may be needed to refine treatment strategies for this specific neuroblastoma subtype.
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.