SHB and hereditary pheochromocytoma-paraganglioma: Systemic chemotherapy using a combination of cyclophosphamide, vincristine, doxorubicin, and dacarbazine is also used for patients with unresectable and rapidly progressive pheochromocytoma, especially in patients with high tumor burden or many bony metastases [34], with a higher efficacy in patients with SHB mutation [35].