Application of the International Neuroblastoma Risk Group Staging System 2009 (Inrgss 2009) in Diagnosis and Prognosis of Childhood Neuroblastoma

Abstract

Background: Neuroblastoma is the most common extracranial solid tumor of childhood. INRGSS 2009 divides patients into stages L1, L2, M, Ms, from which, in combination with histopathology, molecular biology, each patient will be divided into risk groups from which to guide prognosis and suitable treatment. Objectives: Describe the clinical and laboratory features of neuroblastoma in children; distribution of pediatric neuroblastoma stages according to the INRGSS 2009 classification and its value in predicting survival outcomes. Methods: This was a cross-sectional study combined with retrospective and prospective. Patients were diagnosed with neuroblastoma and classified according to INRGSS 2009 at the Pediatric Center, Hue Central Hospital. Results: The age at the time of diagnosis ranged from 1 day old to 9 years old, with a mean of 35.4 ± 25.5 months. Regarding primary tumor, 73.5% of cases were found in the abdomen, the mediastinum region (22.4%), an undetermined region (4.1%). Systemic symptoms, local symptoms and symptoms caused by metastasis were frequently reported. At the time of diagnosis, 73.5% of the patients had distant metastases (stage M or MS). The EFS of the whole group at 12 and 24 months old were 86.3 % and 64.7% respectively. The OS was 77.9% at 24 months old. Most of these patients had a progressive disease: 6.1% were in stage L1; 20.4% were in stage L2; 69.4% were in stage M; and 4.1% were in stage MS. Estimated mean of event-free survival for L1 was 38.7 ± 3.5 months, L2 was 40.5 ± 4.2 months and M was 28.0 ± 2.5 months (p < 0.05). Conclusions: Most neuroblastoma tumors are abdominal and have distant metastases. LDH and ferritin elevations are prevalent. Neuroblastoma outcomes can be predicted using the INRGSS 2009 classification.