Congenital intrathoracic neuroblastoma presenting as persistent pulmonary hypertension in a newborn

doi: 10.1007/s12519-011-0288-2 ©Children's Hospital, Zhejiang University School of Medicine, China and Springer-Verlag Berlin Heidelberg 2011. All rights reserved. A female infant, weighing 2740 g, was vaginally born at 42 weeks of gestation. The Apgar scores were 7 and 8 at 1 and 5 minutes, respectively. After birth, the infant immediately developed respiratory distress, and required conventional ventilation, with an initial peak positive pressure of 15 cmH2O, positive end-expiratory pressure of 4 cmH2O, frequency of 40/min, and supplemental oxygen of 100%. A chest radiograph taken within the first hour following birth revealed retained fetal lung fluid with homogenous round opacity at the right upper lung (Fig. A). At 3 hours of life, she developed severe hypoxemia with 75% of pre-ductal saturation (SpO2) and 64% of post-ductal SpO2, leading to a clinical diagnosis of persistent pulmonary hypertension of the newborn (PPHN). Her respiration support was substantially changed to high frequency oscillatory ventilation with a mean airway pressure of 12 cmH2O, delta-pressure of 40 cmH2O, frequency of 10 Hz, and supplemental oxygen of 100%. The results of an arterial blood gas taken at 6 hours of life were PaCO2 79 mmHg, PaO2 54 mmHg, and pH 7.12. Serial chest X-rays at 24 hours of life revealed disappeared fetal lung fl uid and the round opacity became more obvious (Fig. B). At 3 days of life, she was weaned from supplemental oxygen, inotropic therapy, and sedative drugs, and respiratory support was switched to conventional ventilation at her 4 days of life. Computed tomography demonstrated a posterior mediastinal mass with faint internal calcifications and tracheal displacement (Fig. C). Ventilator dependency suspected to be secondary to an airway obstruction led to the attending physician performing a thoracotomy on the 12th day, which revealed a tumor which was later removed. Histology of the tumor showed small round cells in a pseudorosette formation, compatible with a poorly differentiated neuroblastoma (Fig. D). Unfortunately, the patient died of septicemia 2 months later. Congenital neuroblastoma is the most common malignant solid tumor in newborns, accounting for 54% of all neonatal malignancies. However, intrathoracic lesion is very rare in this age group. All reported neonatal intrathoracic neuroblastomas have presented with respiratory symptoms and required respiratory Congenital intrathoracic neuroblastoma presenting as persistent pulmonary hypertension in a newborn