STAGE IVS NEUROBLASTOMA - A CLINICAL, HISTOLOGICAL, AND BIOLOGICAL ANALYSIS OF 45 CASES

This study reports clinicopathologic and biological analysis of 45 sta ge IVS neuroblastomas (IVS-NBs). All IVS-NB cases had small or undetec table primary tumors associated with disease involving the liver, skin , or bone marrow. Their ages at diagnosis ranged from 1 day to 12 mont hs, with a median age of 4 months. The primary sites were adrenal in 3 4 cases, retroperitoneum in two, mediastinum in one, and unknown in ei ght; the liver was involved in 24 of 31 cases, the bone marrow in 11 o f 27, and the skin in 4 of 29. According to the histopathological eval uation (Shimada classification), 43 IVS-NB tumors had a favorable hist ology, and 2 cases showed an unfavorable histology. Three patients die d of progressive disease and six of indirect tumor death. Among these three deceased cases with tumor progression, two cases showed unfavora ble histology, and one case had amplified N-myc (20 copies). These dat a suggest that most IVS-NB can be described as a tumor presenting char acteristic dissemination pattern in the younger age group, having a fa vorable histology and showing an excellent outcome of the patients. Ho wever, poor prognosis group in stage IVS does exist, and this group co uld be detected histopathologically and biologically. Copyright (C) 19 96 by W.B. Saunders Company