New advances in pheochromocytoma and paraganglioma--the 5th international symposium on pheochromocytoma and paraganglioma
10.3760/cma.j.issn.1000-6699.2018.06.016
- VernacularTitle:嗜铬细胞瘤和副神经节瘤研究新进展——记第五届国际嗜铬细胞瘤和副神经节瘤大会
- Author:
Lin LU
1
;
Shi CHEN
;
Zhengpei ZENG
Author Information
1. 100730 中国医学科学院,北京协和医学院,北京协和医院内分泌科,国家卫生和计划生育委员会内分泌重点实验室
- Keywords:
Pheochromocytoma;
Paraganglioma;
Molecular biology;
Molecular imaging;
Treatment
- From:
Chinese Journal of Endocrinology and Metabolism
2018;34(6):532-536
- CountryChina
- Language:Chinese
-
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.
