Treatment of Pheochromocytoma in Metastatic PPGL That Is MIBG Nonavid, Has Mixed Tumors, Contraindication to MIBG, or Is Rapidly Progressive

Metastatic pheochromocytoma/paraganglioma (mPPGL) poses a specific therapeutic challenge when MIBG-based approaches are not suitable. This page describes the relevant clinical scenario and the general direction of the evidence-based protocol.

Clinical Scenario

This protocol is indicated for patients with metastatic pheochromocytoma or paraganglioma (mPPGL) who present in one or more of the following situations: tumors that are MIBG nonavid (not expressing the noradrenaline transporter), mixed tumor types, a contraindication to MIBG therapy (such as bone marrow suppression due to bone metastases), or rapidly progressive disease.

Treatment Approach (Partial Overview)

For patients who meet these criteria, a tyrosine kinase inhibitor (TKI)-based strategy is the therapeutic direction supported by current evidence. Prior to starting treatment, patients require preparation with antihypertensive agents to manage associated cardiovascular risks; ongoing dose management may also be required depending on blood pressure response.

The complete protocol — including agent selection, sequencing, and full clinical management — is available below.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1097/MPA.0000000000001792

The TKIs could be a therapeutic option for patients with mPPGL, especially for those with tumors that do not express the noradrenaline transporter (MIBG nonavid), mixed tumors, and patients with contraindications for MIBG therapy (ie, bone marrow suppression due to bone metastases) or for any patients with rapid progression (consensus).

Axitinib, cabozantinib, lenvatinib, pazopanib, and sunitinib are TKIs evaluated in phase 2 clinical trials for patients with mPPGL.

Because of associated cardiovascular toxicities with TKIs, patients must be prepared with α-and β-blockers and other antihypertensives before treatment is started.

The dose of the TKI may need to be adjusted down in response to high BP.

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