Clinical Scenario

This protocol addresses patients with metastatic pheochromocytoma or paraganglioma (mPPGL) whose disease is negative on MIBG imaging but positive on somatostatin receptor (SSTR) PET. This distinct imaging profile places these patients outside the standard MIBG-based treatment pathway and calls for a different therapeutic approach.

Treatment Approach (Overview)

A targeted radionuclide therapy acting on somatostatin receptors is among the strategies considered for this population. Given the limited evidence base, participation in a clinical trial is recommended where available. Pre-treatment blood pressure control is an important clinical consideration before initiating therapy.

References

DOI: 10.1097/MPA.0000000000001792

177Lu-DOTATATE should be considered in patients who are negative on MIBG imaging, but positive on SSTR PET.
Preliminary data suggest potential clinical efficacy of 177Lu-DOTATATE in a subgroup of patients with mPPGL; however, we suggest participation in a clinical trial if this therapy is considered (consensus).
Similar to 131I-MIBG treatment, acute catecholamine crises can occur with 177Lu-DOTATATE therapy, and patients' hypertension should be medically managed before treatment.

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Treatment of Metastatic Pheochromocytoma / Paraganglioma Negative on MIBG Imaging and Positive on SSTR PET

References