Pheochromocytoma
ICD-10 E27.5 · ICD-11 5A75

Managing Metastatic Pheochromocytoma/Paraganglioma with Bone Metastases

Bone involvement is among the most frequent sites of spread in metastatic pheochromocytoma/paraganglioma (mPPGL), and its presence has direct implications for how the disease is managed alongside systemic treatment.

The skeletal system is one of the most common sites of metastatic disease in mPPGL, occurring in at least 60–70% of patients, and is limited to bone alone in roughly 20% of patients. When bone metastases are present — particularly in weight-bearing sites — skeletal management becomes a central part of the overall treatment plan.

The structured protocol addresses skeletal protection through bone-targeted agents, with guidance on patient selection, pre-treatment evaluation, and the role of radiation for specific bone sites. Full regimen details, selection criteria, and sequencing are in the complete protocol.

References
DOI: 10.1097/MPA.0000000000001792
  • The skeletal system is 1 of the most common sites of metastatic disease in mPPGL, occurring in at least 60% to 70% of patients and limited to bone alone in 20% of patients.
  • If there is bony disease, consider bone-targeted agents such as denosumab 120 mg subcutaneous every 3 months or zoledronic acid 4 mg intravenous every 3 months and continued during disease progression (consensus).
  • Because stopping denosumab may accelerate bone loss, we recommend its use only in those with renal function limiting the use of zoledronic acid.
  • Thus, ensuring calcium and vitamin D levels are at target and completing a dental evaluation before the initiation of bone-targeted agents are recommended.
  • If bone metastases are in weight-bearing bones, we recommend radiation to those sites for stability (significant majority).
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