Management and Outcomes of Children with Stage 4S (MS) Neuroblastoma: A Single-Center Experience from a Resource-Challenged Nation.

OBJECTIVES

To demonstrate the clinical profile, management options, and outcomes of children with stage 4S neuroblastoma (NB 4S) diagnosed at a tertiary care center in a resource-challenged nation. The authors also intend to highlight the factors associated with an unfavorable prognosis in this series of patients.

METHODS

The archives for children with NB 4S, diagnosed over a 24-y period (January 1996-December 2019), were retrospectively retrieved. Data on patient characteristics, management protocols, oncologic outcomes, and overall survival (OS) were reviewed. Multivariate logistic- regression analysis was performed to identify the factors independently predicting unfavorable outcomes.

RESULTS

A total of 22 children (59% males) were included. Adrenal was the most common (82%) primary site. Liver involvement (100%), bone marrow infiltration (23%), and subcutaneous nodules (9%) were observed upon evaluation. Management involved supportive treatment (22%), chemotherapy only (41%), chemotherapy and tumor excision (28%). Ventral hernia was created in two children (9%) due to abdominal compartment syndrome (ACS). Four children died (4/22; 18%) due to ACS (n = 2) and refractory coagulopathy (n = 2). There were no recurrences and all survivors were disease-free. The 5-y OS was 81.8% with a median follow-up duration of 31 mo (range 9 mo-22 y). Age < 2 mo (p = 0.002), respiratory distress at presentation (p < 0.001), and chemotherapy nonresponsiveness (p < 0.001) were significantly associated with mortality. All three factors were independent predictors of mortality.

CONCLUSION

Children with NB 4S have a favorable outcome with 5-y OS of 81.8%. Age < 2 mo, respiratory distress at presentation, and chemotherapy nonresponsiveness are independent predictors of a poor outcome.