OBJECTIVE Adrenal ganglioneuromas are rare, differentiated, neuroblastic tumors that originate from the peripheral sympathetic nervous system. Because of their rarity, information is limited, derived from small cases series. Our objective was to characterize this tumor and provide help for its management. METHODS A retrospective multicenter analysis of adrenal ganglioneuromas 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 indeterminate for 9.2% of patients (n = 9/98). CT imaging, performed on 96 patients, revealed large tumors (median 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 nonfunctioning, without benign imaging features. Although the duration of follow-up was limited in our series, no recurrence was identified. A review of the literature confirms the absence of postoperative recurrence. Based on all available 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.

中文翻译：

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肾上腺神经节神经瘤：来自 COMETE 网络的 104 例病例的回顾性多中心研究。

目的 肾上腺神经节神经瘤是起源于周围交感神经系统的罕见、分化、成神经细胞肿瘤。由于它们的稀有性，信息有限，来源于小案例系列。我们的目标是描述这种肿瘤的特征并为其管理提供帮助。方法 对来自 COMETE 网络的 20 个法国中心和一个比利时中心的肾上腺神经节神经瘤进行回顾性多中心分析。结果 在确定的 104 例病例中，59.6% 为女性（n = 62/104），诊断时的中位年龄为 29 岁，其中 24 例为儿科病例。60.6% (n = 63/104) 是偶发瘤。90.8% 的病例 (n = 89/98) 中的神经节神经瘤是非分泌性肿瘤，而 9.2% 的患者 (n = 9/98) 的术前激素评估不确定。CT成像，对96名患者进行，在 98.1% (n = 52/53) 和 64.6% (n = 31/48) 的病例中发现了非对比密度 > 10 Hounsfield 单位的大肿瘤（中位直径为 50 mm）和钙化。在 26.7% (n = 8/30) 和 42.2% (n = 19/45) 的肿瘤中分别观察到 123I-MIBG 闪烁显像和 18F-FDG-PET/CT 摄取增加。所有 104 名患者均接受了手术治疗。在进行影像学随访的 42 名患者中未观察到复发（平均 29.6 个月，中位数 18 个月 (4-156)）。结论 肾上腺神经节神经瘤为大肿瘤，多无功能，无良性影像学特征。尽管我们的系列随访时间有限，但未发现复发。文献回顾证实没有术后复发。根据所有可用数据，在没有特殊情况的情况下（遗传形式、