Summary
Pheochromocytoma and paraganglioma (PCPG/PPGL) are rare neuroendocrine tumors that arise from chromaffin cells and can occur in the adrenal glands or in extra-adrenal sites such as the thorax, abdomen, pelvis, bladder, and head and neck.
Surgery remains the only curative option for localized disease, while advanced cases rely on individualized use of alpha and beta blockade, chemotherapy, radionuclide therapy, PRRT, and targeted therapies.
The approval of Merck’s belzutifan (WELIREG) in May 2025 was a major milestone because it became the first FDA-approved oral therapy for advanced PPGL and added a new precision-based option for eligible patients.
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