Paediatric Tumours of Neuroendocrine/Peripheral Neuroectodermal Origin

Abstract

The neoplasms arising from the neural crest-derived cells in the adrenal medulla or extra-adrenal paraganglia include peripheral neuroblastic tumours, pheochromocytoma and paraganglioma. Peripheral neuroblastic tumours (neuroblastoma, ganglioneuroblastoma and ganglioneuroma) account for 7–10% of all tumours in childhood. In these tumours, the MYCN oncogene status, extension of the disease and age are the most relevant prognostic factors. The 5-year survival probability ranges from >95% for patients with nonmetastatic resectable tumour and absence of MYCN amplification to <20% for patients with metastatic disease and MYCN-amplified tumour.