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← Pheochromocytoma and Paraganglioma

HEALTH PROFESSIONAL · SOURCE READING

Chemotherapy

Source: Pheochromocytoma and Paraganglioma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.

Source updated: November 1, 2024 · Captured 2026-09-09.

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Context: Treatment of Metastatic Pheochromocytoma / Treatment Options for Metastatic Pheochromocytoma / Palliative therapy

Chemotherapy has not been shown to improve survival in patients with metastatic pheochromocytoma; however, chemotherapy may be useful for symptom palliation.

The best-established chemotherapy regimen is a combination of cyclophosphamide, vincristine, and dacarbazine (the Averbuch protocol).[1]

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Evidence (chemotherapy):

A nonrandomized single-arm trial included 18 patients with metastatic malignant pheochromocytoma or paraganglioma. Patients were treated with a combination of cyclophosphamide, vincristine, and dacarbazine.[2]

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A nonrandomized single-arm trial included 18 patients with metastatic malignant pheochromocytoma or paraganglioma. Patients were treated with a combination of cyclophosphamide, vincristine, and dacarbazine.[2]

After 22 years of follow-up, the complete response rate was 11%, the partial response rate was 44%, the biochemical response rate was 72%, and the median survival was 3.3 years.[2][Level of evidence C3]

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A retrospective study showed a therapeutic benefit of temozolomide in patients with metastatic pheochromocytoma or paraganglioma. Fifteen consecutive patients with metastatic pheochromocytoma or paraganglioma were enrolled; 10 (67%) had an SDHB variant. The mean dose intensity of temozolomide was 172 mg/m2 daily for 5 days every 28 days.[3]

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A retrospective study showed a therapeutic benefit of temozolomide in patients with metastatic pheochromocytoma or paraganglioma. Fifteen consecutive patients with metastatic pheochromocytoma or paraganglioma were enrolled; 10 (67%) had an SDHB variant. The mean dose intensity of temozolomide was 172 mg/m2 daily for 5 days every 28 days.[3]

The median progression-free survival was 13.3 months after a median follow-up of 35 months. Of the 15 patients, 5 (33%) had a partial response, 7 (47%) had stable disease, and 3 (20%) had progressive disease.[3][Level of evidence C3]

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Several other chemotherapy regimens have been used in small numbers of patients, but the overall results were disappointing.[4,5]

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Publication references

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Preserved source evidence · Not medical advice