ESPEYB21 8. Adrenals Important for Clinical Practice (8 abstracts)
8.10. Management of phaeochromocytoma and paraganglioma in patients with germline SDHB pathogenic variants: an international expert consensus statement
Taïeb D , Nölting S , Perrier ND , Fassnacht M , Carrasquillo JA , Grossman AB , Clifton-Bligh R , Wanna GB , Schwam ZG , Amar L , Bourdeau I , Casey RT , Crona J , Deal CL , Del Rivero J , Duh QY , Eisenhofer G , Fojo T , Ghayee HK , Gimenez-Roqueplo AP , Gill AJ , Hicks R , Imperiale A , Jha A , Kerstens MN , de Krijger RR , Lacroix A , Lazurova I , Lin FI , Lussey-Lepoutre C , Maher ER , Mete O , Naruse M , Nilubol N , Robledo M , Sebag F , Shah NS , Tanabe A , Thompson GB , Timmers HJLM , Widimsky J , Young WJ Jr , Meuter L , Lenders JWM & Pacak K
Nat Rev Endocrinol. 2024; 20(3): 168-184. https://pubmed.ncbi.nlm.nih.gov/38097671/
Brief Summary: The management of phaeochromocytomas and paragangliomas in patients with pathogenic variants of succinate dehydrogenase complex iron sulfur subunit B ( SDHD ) gene is complex. An international group of experts performed a critical review of the evidence to produce this consensus statement to assist clinical decision-making.
Comment: Pathogenic variants in succinate dehydrogenase complex iron sulfur subunit B ( SDHD ) gene are known causes of phaeochromocytomas and paragangliomas (PPGLs) (1). Their life-time disease penetrance is relatively high, and the recurrence rate for SDHB PPGLs is also high (2). Furthermore, these PPGLs are prone to aggressive behavior. Approximately 1/3 of patients develop metastases and have a predisposition to develop other tumors. Due to the complexity in clinical management of patients with SDHB PPGLs, the authors developed this consensus statement after reviewing the available evidence.
The authors comprised experts in PPGLs from a variety of countries, practice settings and disciplines. They reviewed published evidence and produced 29 graded recommendations on the clinical management of patients with an existing PPGL. The recommendations range from pre-operative work up to surveillance and different treatments. The authors strongly recommend that all major treatment and management decisions of patients with SDHB PPGLs should be carried out in an expert, interdisciplinary team setting. This approach facilitates personalized tailoring of patient management in specific clinical situations, including plans for individualized surveillance and follow-up. This consensus will facilitate standardized high-quality care for patients with PPGL due to SDHB pathogenic variants.
References: 1. Neumann HP, Pawlu C, Peczkowska M, Bausch B, McWhinney SR, Muresan M, Buchta M, Franke G, Klisch J, Bley TA, Hoegerle S, Boedeker CC, Opocher G, Schipper J, Januszewicz A, Eng C; European-American Paraganglioma Study Group. Distinct clinical features of paraganglioma syndromes associated with SDHB and SDHD gene mutations. JAMA. 2004; 292(8): 943-51. 2. Assadipour Y, Sadowski SM, Alimchandani M, Quezado M, Steinberg SM, Nilubol N, Patel D, Prodanov T, Pacak K, Kebebew E. SDHB mutation status and tumor size but not tumor grade are important predictors of clinical outcome in pheochromocytoma and abdominal paraganglioma. Surgery. 2017; 161(1): 230-239.
Volume 21
Article tools
My recent searches
My recently viewed abstracts
Authors
ESPE Yearbook of Paediatric Endocrinology
ISSN 1662-4009 (Online)
© Bioscientifica 2024 |
Privacy policy |
Cookie settings
BiosciAbstracts
Bioscientifica Abstracts is the gateway to a series of products that provide a permanent, citable record of abstracts for biomedical and life science conferences.
Find out more