Surgical treatment and management of syndromic paraganglioma. The experience of a referral center

BACKGROUND: Paragangliomas (PGL) are neuroendocrine tumors arising from neural crest-derived chromaffin cells in the parasympathetic ganglia or in sympathetic ganglia located in the adrenal glands (Pheochromocytoma, PHEO), or extra-adrenal location and around 30-40% of PGL can be hereditary (MEN2, Von Hippel Lindau syndrome, von Reklingausen syndrome and Pheochromocytoma-Paraganglioma syndrome). METHODS: We retrospectively evaluate 127 patients with PHEO/PGL treated at our Institution from 1992 to 2018 and report our experience, focusing on different surgical treatment based on hereditary syndrome. Genetic analysis was performed in patients with features suspected for hereditary syndrome to search genetic mutations of RET, VHL, SDHD and SDHB. RESULTS: Hereditary syndrome was diagnosed in 30 patients (7 patients affected by MEN2A, 6 VHL, 9 NF1, 2 PGL-1, and 6 PGL-4). 17 patients with monolateral PHEO underwent adrenalectomy (laparoscopic approach since 1998); 8 patients with bilateral adrenal lesions underwent bilateral adrenalectomy with cortical-sparing approach of almost one gland. CONCLUSIONS: Syndromic PGLs are rare conditions; surgery is the treatment of choice. Adrenal PGL requires laparoscopic adrenalectomy with transperitoneal lateral approach as gold standard. For genetic syndrome such as MEN2 and VHL, laparoscopic cortical sparing adrenalectomy of at least one gland should be considered.