Metabolic activity and clinical features of primary ganglioneuromas

BACKGROUND. Ganglioneuroma (GN) is considered by most to be a benign tumor of neural crest origin. It may evolve from differentiating neuroblastoma or may be diagnosed as primary ganglioneuroma. The rarity of this tumor and t he lack of understanding of its biology often lead to inaccurate diagnosis and treatment. METHODS. The authors analyzed clinical features and biologic behavior of pr imary ganglioneuroma in 149 patients who were registered with but were not part of the national neuroblastoma trials. Data included age and symptoms a t diagnosis, gender, secretion of catecholamines, histology, treatment, and outcome, whenever available. RESULTS. Patients with primary ganglioneuroma were significantly older than patients with neuroblastoma. Median age at diagnosis was 79 months compare d with 16 months (P < 0.0001). Ganglioneuroma were equally distributed betw een males and females (1.13:1). A preference of thoracic (41.5%) and abdomi nal, nonadrenal tumors (37.5%) was observed compared with adrenal GN (21%). At diagnosis, thoracic tumors appeared larger than nonthoracic ones. Local lymph node metastases occurred in two patients. On ganglioneuroma had meta stasized to soft tissues. I-123-mIBG scintigraphy detected mIBG uptake at t umor site in 57% of the GN tumors. Levels of catecholamines in plasma and/o r urine were increased in 39%. Slight immaturity of ganglion cells was obse rved in 93% of all ganglion-neuroma tumors. None of the 22 tumors analyzed exhibited MYCN gene alterations. Although 12 patients had macroscopic resid uals, no tumor progression or recurrence was observed in a median follow-up of 25 months. CONCLUSIONS. Ganglioneuroma may present with metabolic activity such as inc reased secretion of catecholamines and/or mIBG uptake. There are no specifi c diagnostic signs or symptoms discriminating ganglioneuroma and neuroblast oma tumors. Therefore, ganglioneuroma requires tissue investigation for dia gnosis. Prognosis after surgical resection without further therapy seems to be excellent. (C) 2001 American Cancer Society.