Clinical Characteristics and Outcome of High Risk Neuroblastoma Treated at Sohag Cancer Center, Egypt

Background objectives: Neuroblastoma is the most common extracranial solid 
tumor in children. Nearly half of them are high risk at diagnosis according to the 
International Neuroblastoma Risk Group Staging System (IRGSS). Patients with 
high risk tumors have dismal outcome with 5 years survival rate only 50% 
despite aggressive therapy. The study aimed to present the clinical 
characteristics and outcome in patients diagnosed with high risk neuroblastoma 
(HR-NB) during the period from January 1st, 2014, to December 31st, 2019 and 
their follow up data were collected till 30th June 2020.
Methods: A retrospective study was conducted on a group of 34 patients 
diagnosed with HR-NB and treated at Sohag Cancer Center by two different 
chemotherapy protocols. 
Results: The study group had a median age of 33 months, with 16 patients 
(47%) being males and 18 patients (52.9%) females. Most of patients (79.4%) 
aged ≥1.5 years. The main presenting symptoms included abdominal mass 
(88%), pallor (47%) and abdominal pain (45.2%). All patients presented with 
advanced-disease stage, with 91.1% had stage IV and 8.8% had stage III. 
Abdomen was the most common primary site for the disease reported in 88.2%. 
Nineteen patients treated according to the OPEC/OJEC protocol (55.9%) (group
1) and fifteen patients received the High risk COG protocol (group 2). Local 
control by surgery was feasible in 22 patients. At a median follow up of 29 
months, the 3-year OS and EFS were 47 % and 35.3% respectively which were 
significantly affected by MYC N gene status, protocol of chemotherapy used, 
receiving radiotherapy and having ABMT. Myelosuppression was the main 
treatment related toxicity reported in all patients.
Conclusion: The study's findings align with similar research on HR-NB 
treatment considering very poor prognosis of patients with HR-NB. Using less 
aggressive treatment protocol as with OPEC/OJEC showed better survival and 
less morbidity among patients in this study. Also, MYC N gene status, receiving 
radiotherapy and having ABMT were significantly affecting disease outcome 
and survival of patients with HR-NB.