Adrenal ganglioneuromas: A retrospective multicentric study of 104 cases from the COMETE network

Objective: Adrenal ganglioneuromas are rare, differentiated, neuroblastic tumors that originate from the peripheral sympathetic nervous system. Because of their rarity, informatio n is limited, derived from small cases series. Our objective was to characterize this tumor and provide help for i ts management. Methods: A retrospective multicenter analysis of adrenal ganglioneuroma s from 20 French centers belonging to the COMETE network and one Belgian center. Results: Among the 104 cases identified, 59.6% were women ( n = 62/104), median age at diagnosis was 29 years, with 24 pediatric cases. 60.6% (n = 63/104) were incidentalomas. Ganglioneuromas were non-secreting tumors in 90.8% of cases (n = 89/98), whereas the preoperative hormonal evaluation was indet erminate for 9.2% of patients (n = 9/98). CT imaging, performed on 96 patients, revealed large tumors (media n diameter of 50 mm) with a non-contrast density > 10 Hounsfield units in 98.1% (n = 52/53) and calcifications in 64.6% of cases (n = 31/48). Increased uptake on 123I-MIBG scintigraphy and 18F-FDG-PET/CT was observed in 26.7% (n = 8/30) and 42.2% (n = 19/45) of the tumors, respectively. All 104 patients underwent surgery. No recurrence was observed among the 42 patients who had an imaging follow-up (mean 29.6 months, median 18 months (4-156)). Conclusion: Adrenal ganglioneuromas are large tumors, mostly nonfunctionin g, without benign imaging features. Although the duration of follow-up was limited in our series, n o recurrence was identified. A review of the literature confirms the absence of postoperative recurrence. Based on all a vailable data, in the absence of special circumstances (genetic form, uncertain histological diagnosis), long-term follow-up is not necessary after complete surgery for patients with an adrenal ganglioneuroma.