Perinatal neuroblastoma: a case report of pepper syndrome

Bárbara Ribeiro Aguiar, Department of Pediatrics, Loures, Portugal
Joana Ribeiro, Department of Obstetrics and Gynaecology, Hospital Beatriz Ângelo, Loures, Portugal
Diana Martins, Department of Obstetrics and Gynaecology, Hospital Beatriz Ângelo, Loures, Portugal
Sara Noéme Prado, Department of Pediatrics, Loures, Portugal
Hugo Cavaco, Department of Pediatrics, Loures, Portugal

Introduction: Perinatal neuroblastoma is the most common malignant tumor in the neonatal period. Stage MS occurs in children under the age of 12 months and is a subgroup of localized primary tumors, with metastases limited to the liver, skin or bone marrow. It is also characterized by a high incidence of spontaneous regression and an excellent survival rate. Pepper syndrome is extremely rare in the neonatal period and results from massive hepatic infiltration by neuroblastoma in advanced stages (M and MS), conditioning respiratory compromise, liver failure and coagulopathy. Case report: This case presents this rare form of neonatal neuroblastoma, with rapid metastatic growth and a guarded prognosis due to mechanical complications characteristic of Pepper syndrome. Discussion: Despite the adoption of chemotherapy and intensive support therapy, rapid metastatic growth resulted in progressive worsening with liver failure, peri-ventricular hemorrhage and death.

Keywords: Newborn. Neonatal tumors. Perinatal neuroblastoma. Pepper syndrome. Case report