Endocrine Abstracts

Introduction: Adrenal ganglioneuroma (AGN) is a rare (about 470 described cases), benign tumor arising from the neural crest tissue. These tumors are usually asymptomatic and incidentally detected. They have variable radiological features that often raise suspicion of malignancy. AGN are diagnosed at a mean age of 50 years, have no gender preference and are rarely associated with genetic syndromes.

Aim: The presentation of two male patients, 24 and 53 years old with adrenal incidentalomas and a final diagnosis of AGN.

Case 1: A 24-year-old male patient, with a history of asthma, was referred for evaluation of a left adrenal tumor incidentally discovered on a chest CT performed due to an exacerbation of his respiratory symptoms. The tumor, measuring 1.4 cm, had smooth borders but showed increased density (30 HU). On MRI, the tumor showed mild signal enhancement in T2 sequences and poor enhancement with delayed uptake of paramagnetic substance. Clinical and laboratory workup of the patient were normal. The patient was normotensive and testing for cortisol, metanephrines and adrenal androgens (DHEAS, 17OHPRG), no functionality was detected. The patient underwent a laparoscopic adrenalectomy, the histological examination of which showed morphological features of AGN.

Case 2: A 53-year-old male patient with no previous comorbidities was diagnosed with a large incidentaloma on the right adrenal gland, during a diagnostic work up for fever of unknown etiology. On CT, the incidentaloma had a size of 11×7.5×6.5 cm, high density (>30 HU) and very low absolute and relative contrast media washout. Clinical examination of the patient revealed no signs of hypercortisolism or other pathological findings. Haematological and biochemical tests were normal. Similarly, hormonal workup exclude hypersecretion (normal circadian cortisol rhythm (morning 9.8 μg/dl, evening 1.6 μg/dl), normal 24-hour metanephrines fractions [normetanephrine =261 μg/24 h (<780), metanephrines =41 μg/24 h (<375)] and normal adrenal androgens DHEAS and 17OHPRG. Due to the size of the tumor and the imaging characteristics, which were not compatible with an adrenal adenoma, a retroperitoneal endoscopic right adrenalectomy was performed. Histological examination revealed an AGN with scattered microcalcifications without evidence of necrosis and atypia. The patient, after 4 years of follow-up, remains disease free.

Conclusion: AGNs usually have a benign course. Nevertheless, their imaging features guide patients to their surgical removal to establish a definitive diagnosis and determine prognosis. Age of diagnosis ultimately may not play a decisive role in the differential diagnosis of AGN.