Presentation and outcomes of adrenal ganglioneuromas: A cohort study and a systematic review of literature

Abstract

Objective To describe the presentation and outcomes of patients with adrenal ganglioneuromas (AGNs). Design Single‐centre retrospective cohort study (1 January 1995 to 31 December 2019) and systematic review of literature (1 January 1980 to 19 November 2019). Patients Diagnosed with histologically confirmed AGN. Measurements Baseline clinical, imaging and biochemical characteristics, recurrence rates and mortality. Subgroup analysis was performed on tumours with histologic elements of ganglioneuroma and pheochromocytoma (ie composite tumours). Results The cohort study included 45 patients with AGN, 20 (44%) of which had composite tumours. Compared to pure AGN, patients with composite tumour were older (median age, 62.5 vs. 35 years, p < .001), had smaller tumours (median size, 3.9 vs. 5.7 cm, p = .016) and were discovered incidentally less frequently (65% vs. 84%, p = .009). No recurrences or ganglioneuroma‐specific mortality occurred during follow‐up (range, 0–266 months). The systematic review included 14 additional studies and 421 patients. The mean age of diagnosis was 39 years, and 47% were women. AGNs were discovered incidentally in 72% of patients, were predominantly unilateral (99%) and had a mean diameter of 5.8 cm and an unenhanced computed tomography (CT) attenuation of −118 to 49 Hounsfield units (HU). On imaging, 69% of AGNs were homogenous, 41% demonstrated calcifications, and 40% were lobulated. Conclusions AGNs are rare benign tumours that present with variable imaging features including large size, unenhanced CT attenuation >20 HU, calcifications and lobulated shape. Imaging characteristics can assist in establishing a diagnosis and avoiding an unnecessary adrenalectomy. The association of pheochromocytomas with AGNs is frequent. Diagnosis should include biochemical testing.

Adrenal ganglioneuroma resected for suspicious malignancy: multicenter review of 25 cases and review of the literature

“…An AGN as demonstrated on a CT scan is homogenous and of low attenuation in the precontrast phase, mild attenuation in the postcontrast phase, and sometimes either homogenous or heterogeneous in appearance in the ate phase [ 3 11 ]. This pattern was repeatedly observed by us as well as by others [ 4 5 6 8 9 12 ]. Calcification is another indication for AGN seen on CT; however, its prevalence widely varies between studies from 11.1% [ 2 ] to as high as 82.4% [ 3 ].…”

“…A male-to-female ratio of 2:1 has been reported for adrenomedullary tumors [ 15 ]; however, an equal ratio for both sexes has been found in the present study as well as in other studies [ 9 14 ]. AGN has been reported to accompany hereditary familial syndromes, such as NF1 and NF2 [ 1 2 14 ], and 1 study also reported a single AGN case with an associated multiple endocrine neoplasia 2 syndrome [ 8 ].…”

“…Table 4 summarizes the findings of the present study in comparison to those found in previous large case series (i.e., ≥20 cases). As demonstrated in the present and previous case series [ 1 3 6 7 8 9 10 11 ], the peak incidence of AGN is the 4th–5th decade of life [ 13 14 ].…”

“…The tumor rarely metastasizes or recurs [ 14 ]. No tumor recurrence or death due to AGN was observed in the present study or in any other studies in the literature [ 1 2 3 4 5 6 8 9 10 11 ], although 1 mortality in association with AGN was reported due to CA hypersecretion [ 17 ]. While the overall prognosis is excellent, surgical excision should remain the standard of care due to the inability of radiology to discriminate between AGN from a malignant tumor.…”

“…As of 2021, there are only 5 case series in the English literature reporting over 20 cases and originating in China, South Korea, and the United States [ 3 6 7 8 9 ]; another 3 originating in Italy and China and report 10–17 cases each [ 1 10 11 ] and 4 case series of 6–9 cases each from Canada [ 4 ], Italy [ 2 ], Greece [ 5 ], and Belgium [ 12 ]. The largest studies from the Far East and the United States are single-center experiences and relate to 29—45 cases recorded between 1988 and 2019 [ 3 6 7 8 9 ].…”