Primary pancreatic neuroblastoma: an unusual tumor in infancy

Abstract Fetal imaging has dramatically impacted neonatal care by providing an advanced warning of many different congenital anomalies. The advancements and widespread use of fetal imaging has, however, increased the identification of various incidental findings that is creating new challenges for neonatal diagnosis and treatment. We report such a case where a fetal pancreatic neuroblastoma (NB) was incidentally detected by computed tomographic scan of the maternal abdomen. Primary pancreatic NB is a very uncommon childhood neoplasm that, to our knowledge, has never been previously reported in the English language presenting in either the prenatal or neonatal periods. A 21-year-old woman complaining of acute abdominal pain and carrying a 35 weeks' gestation fetus was referred for computed tomographic scan because of the concern of maternal appendicitis. That scan was inconclusive for appendicitis but did incidentally detect a fetal mass in the general region of the pancreas. At 36 weeks' gestation, the fetus developed signs of distress, which prompted a cesarean delivery. Neonatal workup confirmed the presence of an abdominal mass in the region of the pancreas, but precise anatomic localization was not possible. Also noted on neonatal workup were elevated urinary catecholamines consistent with a hormonal active tumor. These findings prompted an abdominal exploration of this neonate, which revealed a solid tumor contained in the distal pancreas. The mass was managed by an uncomplicated distal pancreatectomy. The neonate fully recovered, and histologic diagnosis revealed NB, whereas the postoperative urine catecholamines normalized. This case underscores the unintended clinical challenges created by widespread fetal imaging, while presenting the first prenatally diagnosed case in the English language medical literature and earliest treated patient with pancreatic NB.