A Huge Mediastino-Retroperitoneal Ganglioneuroma Associated With Multiple Lymph Node Metastases and Pleural Dissemination

Abstract

A right back tumor and a huge abdominal mass were pointed out in a 3-year-and-7-month-old girl. Imaging studies revealed a huge mediastino-retroperitoneal tumor with intrapleural tumor rupture and intraspinal extension. Though histological diagnosis of a biopsied back tumor was benign ganglioneuroma, intrapleural tumor rupture and strongly suspected multiple lymph node metastases in the imaging studies prompted us to give preoperative chemotherapy. After 3 cycles of 91A1 induction chemotherapy neither the size of the tumor nor the elevated urinary VMA and HVA excretion diminished. The main tumor, multiple lymph node metastases and pleural dissemination were subtotally extirpated followed by intraoperative radiation. Histologic diagnosis of the main tumor and all of the lymph node matastases were uniformly ganglioneuroma. The tumor markers normalized postoperatively and tumor recurrence has never been observed. Scoliosis developed 8 years later, necessitating corrective surgery at 10 years. The tumor was considered to have originated as metastatic neuroblastoma in early infancy then gradually maturated to ganglioneuroma. Restricted surgery without intensive chemotherapy and radiation might have been the best treatment of choice.