Adrenal ganglioneuroma: The Padua Endocrine Surgery Unit experience

BACKGROUND: Adrenal ganglioneuroma (AGN) is a rare tumor that originates from the gangliar cells of the sympathetic nervous system. It represents less than 5% of all adrenal masses. AGN occurs as a large mass, with benign behavior and no relevant symptoms and hormonal secretion, but it is often misdiagnosed because the preoperative radiological diagnosis is generally challenging. The aim of the present paper is to report the experience of a tertiary referral academic center regarding the management of AGN and review the relevant literature. METHODS: The demographic, diagnostic, surgical, and pathological findings of 666 consecutive patients who were adrenalectomized at the Padua Endocrine Surgery Unit between 1990 and 2015 were retrospectively reviewed in order to focus on AGN. RESULTS: The pathology confirmed AGN in 10 patients (1.5% of cases; median age 35 years, range 19-73). The diagnosis was incidental and never available before surgery. Eight patients were asymptomatic, two presented lower back and abdominal pain. The mass was non-secreting in all cases. Preoperative imaging techniques were inconclusive about the nature of the mass in all cases. The median size of the tumor was 55 mm (range 30-80). The laparoscopic approach was performed in 60% of cases. No recurrences occurred after surgery at a median follow-up of 10 years (range 1.7-18.2). CONCLUSION: The diagnosis of AGN may be challenging and most often occurs after surgery, since it is frequently confirmed by histological examination. Radical excision may be achieved by laparoscopic adrenalectomy, with a subsequent definitive cure and excellent prognosis at long term follow up.