Fig. 1: Management of metastatic SDHB PPGL.

This figure shows an algorithm for the treatment of metastatic SDHB phaeochromocytoma and paragangliomas (PPGLs). After medical treatment of catecholamine-related signs and symptoms, surgery is considered on an individual basis, depending on several clinical factors that can vary from mild to severe. When considering debulking surgery, note that surgery should be performed if all tumoural lesions can be removed. However, debulking surgery could be considered only in patients with symptoms and signs related to notable catecholamine excess or mass effect. Treatment options depend on the condition of the patient, severity of progression, tumour load and presence of catecholamine-related signs and symptoms. Treatment must be followed up and the patient should be re-evaluated depending on treatment results. Local therapies include radiotherapy, radiofrequency ablation, cryoablation, microwave ablation, embolization, chemoembolization and palliative surgery. [¹³¹I]-MIBG, iodine-131 meta-iodobenzylguanidine; PRRT, peptide receptor radionuclide therapy; TKI, tyrosine kinase inhibitor.