The metastatic pheochromocytomas and paragangliomas treatment landscape has seen notable progress alongside ongoing challenges. Although curative therapies are still lacking, patients benefit from a multidisciplinary care strategy involving debulking surgery, chemotherapy, tyrosine kinase inhibitors, immunotherapy, and radionuclide therapy with 177Lu-DOTATATE.
Around 10% of pheochromocytomas and 40% of paragangliomas are considered malignant. Germline genetic mutations are detected in approximately 30% of patients, with alterations in SDHx genes associated with a higher risk of malignancy. In 2024, the number of newly diagnosed PCPG cases across the 7MM reached app...
