Adult and paediatric patients with pathogenic variants in the gene encoding succinate dehydrogenase (SDH) subunit B (SDHB) often have locally aggressive, recurrent or metastatic phaeochromocytomas and paragangliomas (PPGLs). Furthermore, SDHB PPGLs have the highest rates of disease-specific morbidity and mortality compared with other hereditary PPGLs. PPGLs with SDHB pathogenic variants are often less differentiated and do not produce substantial amounts of catecholamines (in some patients, they produce only dopamine) compared with other hereditary subtypes, which enables these tumours to grow subclinically for a long time. In addition, SDHB pathogenic variants support tumour growth through high levels of the oncometabolite succinate and other mechanisms related to cancer initiation and progression. As a result, pseudohypoxia and upregulation of genes related to the hypoxia signalling pathway occur, promoting the growth, migration, invasiveness and metastasis of cancer cells. These factors, along with a high rate of metastasis, support early surgical intervention and total resection of PPGLs, regardless of the tumour size. The treatment of metastases is challenging and relies on either local or systemic therapies, or sometimes both. This Consensus statement should help guide clinicians in the diagnosis and management of patients with SDHB PPGLs.

编码琥珀酸脱氢酶 (SDH) 亚基 B (SDHB) 的基因存在致病性变异的成人和儿童患者通常患有局部侵袭性、复发性或转移性嗜铬细胞瘤和副神经节瘤 (PPGL)。此外，与其他遗传性 PPGL 相比，SDHB PPGL 的疾病特异性发病率和死亡率最高。与其他遗传亚型相比，具有 SDHB 致病性变异的 PPGL 通常分化程度较低，并且不产生大量儿茶酚胺（在某些患者中，它们仅产生多巴胺），这使得这些肿瘤能够长期亚临床生长。此外，SDHB 致病变异通过高水平的致癌代谢物琥珀酸和与癌症发生和进展相关的其他机制来支持肿瘤生长。结果，发生假性缺氧以及缺氧信号通路相关基因的上调，促进癌细胞的生长、迁移、侵袭和转移。这些因素加上高转移率，支持早期手术干预和完全切除 PPGL，无论肿瘤大小如何。转移瘤的治疗具有挑战性，依赖于局部或全身治疗，或有时两者兼而有之。本共识声明应有助于指导临床医生诊断和管理 SDHB PPGL 患者。