Systemic Treatment of Advanced Metastatic Pheochromocytoma/Paraganglioma with MIBG-Avid Disease

Patients with advanced metastatic pheochromocytoma or paraganglioma (mPPGL) who require systemic therapy and have confirmed uptake on MIBG imaging represent a distinct population for whom a targeted radionuclide approach is indicated.

Clinical scenario

The patient has advanced metastatic pheochromocytoma/paraganglioma requiring systemic therapy, with uptake demonstrated in sites of disease on MIBG imaging. This MIBG-avid status is the defining eligibility criterion for this treatment approach.

Treatment approach — partial overview

High specific activity ¹³¹I-MIBG (iobenguane I 131) targeted radionuclide therapy is indicated for eligible patients with MIBG-avid mPPGL. Adequate blood pressure control and specific pre-treatment pharmacological blockade are required before therapy is initiated. The complete dosing schedule, number of treatment cycles, interval, and full management algorithm are in the structured protocol below.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1097/MPA.0000000000001792

The use of MIBG therapy is indicated for patients with advanced mPPGL requiring systemic therapy and who have uptake in sites of disease on MIBG imaging.
High specific activity ¹³¹I-MIBG should be considered for patients requiring systemic therapy and who have MIBG-avid disease (consensus).
The only prospective registrational trial to date treated hypertensive mPPGL patients with high specific activity MIBG at 296 MBq/kg up to a maximum of 18.5 GBq (500 mCi) in each of 2 treatments 3 to 6 months apart.
As for patients with mPPGL undergoing any systemic therapy, patients should have their BP well controlled before treatment and should be on α/β-blockade at time of treatment.

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