Ganglioneuroblastoma of the posterior mediastinum: a clinicopathologic review of 80 cases

Abstract

The pathology and clinical course of 80 cases of ganglioneuroblastoma (GNB) of the posterior mediastinum are presented. Many neoplasms in this series had previously been classified as differentiating neuroblastomas or immature ganglioneuromas. The prognosis for this group is far better than that for either neuroblastomas or intra-abdominal GNBs. Follow-up was obtained for 72 patients, of whom 55 were followed for 2-23 years. The five-year actuarial survival rate was 88%. Prognosis was related to histologic growth pattern, age, and extent of disease at diagnosis. Stage I tumors were curable by simple excision; children with Stage II tumors treated with surgery and radiation responded as favorably as did those who received adjunctive chemotherapy. In contrast to those associated with late recurrences, the only examples of fatal primary tumors, with one exception, were those patients with Stage IV lesions at the time of initial diagnosis. The therapeutic implications and need for uniform terminology in classifying GNBs are discussed.