New advances in pheochromocytoma and paraganglioma--the 5th international symposium on pheochromocytoma and paraganglioma

Abstract

The 5th international symposium of pheochromocytoma and paraganglioma (PPGL) has been held in September 2017 in Sydney. The conference included the new advances and progresses in the pathogenesis, diagnosis, and treatment of the PPGL. Comprehensive molecular characterization revealed that 95% of PPGL had single or combined germline mutations (27%), somatic mutations (39%), fusion gene (7%) or copy number alterations (89%). PPGL could be divided into kinase signaling subtype, pseudohypoxia subtype, Wnt-altered subtype and cortical admixture subtype. The sensitivity of 68Ga-dotatate-positron emission tomography/computed tomography (PET/CT) to detect the PPGL was quite high, which provided a basis for the treatment of PPGL by 177Lu-dota. The treatment of malignant PPGL included tyrosine kinase inhibitors, 131I-metaiodobenzylguanidine (MIBG), and pembrolizumab. The ultratrace 131I-MIBG, was quite effective for the treatment of malignant PPGL. Although PPGL is a rare and complicated disease, multidisciplinary team and further research will bring more benefits to the early detection and appropriate treatment of PPGL patients. (Chin J Endocrinol Metab, 2018, 34: 532-536) Key words: Pheochromocytoma; Paraganglioma; Molecular biology; Molecular imaging; Treatment