Long clinical benefit achieved in two patients with malignant paraganglioma treated by metronomic cyclophosphamide

ABSTRACT  Treatment of malignant paraganglioma remains a challenge with an overall 5-year survival rate between 34 and 60%. Systemic treatment is recommended in case of unresectable malignant paraganglioma. Recent advances in molecular biology provided rationale to use antiangiogenic agents in this setting. Metronomic cyclophosphamide could be efficient by antiangiogenic effect and immune stimulation with a good safety profile. Here, we report two cases of malignant paraganglioma in frail and symptomatic patients, achieving a long-term clinical benefit with such regimen, after progression or toxicity with sunitinib.