Clinical Characteristics of Peripheral Neuroblastic Tumors in Children: A Single-Center Experience of 43 Cases

Background: The true incidence of peripheral neuroblastic tumors (PNTs) in children is unknown. This study aims to review and analyze clinical data on the diagnosis and management of pediatric PNTs. Methods: Between 2007 and 2016, a total of 43 pediatric patients admitted to our institute with PNTs were reviewed. Results: The series comprised of 23 males and 20 females with a median age of 1.2 years old. Among the 43 PNTs, 26 tumors originated from the abdomen, 13 from the thorax and four from other primary sites. A total of 16 tumors were identified in routine examinations. Abdominal pain and distension were the main clinical manifestations of abdominal PNTs, while coughing was the most frequent presenting symptom of thoracic PNTs. Elevated vanillylmandelic acid level in the urine over 24 hours was observed in 18 neuroblastoma cases and three ganglioneuroblastoma cases. Neuroblastoma was the most common type of PNT that was reported in 30 (69.8%) patients, followed by ganglioneuroblastoma, which was diagnosed in 11 (25.6%) patients. Only 2 (4.6%) patients were diagnosed with ganglioneuroma. A total of 12 cases were stage, six cases were stage II, three cases were stage III, 18 cases were stage IV, and four cases were stage IVs. The overall two year survival rate was 62.9%, which was related to pathological type, Shimada classification, stage, and primary site. Conclusions: Pediatric PNTs have different clinical characteristics and outcomes. Imaging and laboratory data may be useful for the differentiation of PNTs. This study will help pediatric surgeons be aware of the possible manifestations of PNTs in children.