Neuroblastoma. A study of the clinicopathological features influencing prognosis based on the analysis of 54 cases

Abstract

The retrospective analysis of 54 cases of neuroblastoma taken from the files of the Department of Pathology, University of Santiago Hospital, Spain, and the Ludwig-Aschoff Institute of Pathology, University of Freiburg, Germany confirmed the validity and significance of various clinical and histopathological features when trying to establish the prognosis and the proper therapeutic approach in a given case of neuroblastoma. When the age of the patients was compared to survival it was shown that all but three of the patients older than 2 years of age had died from tumor within ten months. In contrast, there was a 37.5% five-year survival rate among patients who were 24 months of age or younger at the time of diagnosis and treatment. The primary tulnor was located in the adrenal gland in 27 cases (50%), in 9 cases (17%) the tumor was retroperitoneal but extra-adrenal, and in the remaining 18 patients (33%) the tumor arose from the paravertebral sympathetic ganglia. Adrenal primaries behaved in an extremely aggresive manner as all but three patients with tilmors at this location were dead within 18 months. Retroperitoneal extra-adrenal neuroblastomas followed an almost equally poor outcome with only one five-year survivor ( 1 1 %). In contrast, 49% of the patients with paravertebral neuroblastoma had survived five years and a further 33% were alive with shorter follow-up. According to histological criteria, there were 6 grade I turnors, 15 grade I1 and 33 grade 111 tumors in our series. All grade I tumors were clinical stage 1 at diagnosis and all are alive 2 to 3 112 years later. Grade I1 tumors were clinical stage 2, 3 or 4 and showed a 46% five-year survival. With the exception of three patients with paravertebral tumors, all patients with grade 111 neuroblastoma were clinical stage 3 or 4 when initially Offprint requests to: Dr. Juan Varela-Duran, Departamento de Anatomia Patologica, Hospital General de Galicia, C/ Galeras s/n, 15702 Santiago de Cornpostela, Spain seen and all were dead from tumor within ten months, with a five-year survival of 9%. It is concluded that the age at diagnosis, location of the primary tumor and histological differentiation, all of which are interrelated, are the most reliable clinicopathological features affecting prognosis and therapy in neuroblastoma.