Endocrine Abstracts

Pheochromocytomas and paragangliomas are rare neuroendocrine chromaffine tumors located in the adrenal or extra-adrenal sites, respectively. Malignant pheochromocytomas or paragangliomas represent 10–20% (MPP) of these patients and are defined by the presence or occurrence of metastasis, in non chromaffin organs. MPP are characterized by their heterogeneous presentation as testified by their variety in primary locations, levels and type of hormone secretions, percentage of genetic disorders and finally frequency of bone metastatic locations. Due to their scarcity, prognostic of MPP have been seldom studied but a hudge heterogeneity in survival has been suggested. SDHB mutation but also the primary location or hormone secretions may constitute critical prognostic parameters. Treatment of MPP has two main goals: the control of hormone-related symptoms and tumor-related burden. In the absence of curative options for advanced MPP patients and randomized trials, the balance between benefits of therapeutic interventions and their safety should be carefully weighed, within skill expert centers and networks, especially in asymptomatic MPP patients with slow rate of progression and low tumor burden. Multiple locoregional options are available to control local progression but also systemic therapies that include meta-iodo-benzyl-guanidine (MIBG) or peptide receptor radionucleide therapy but also, dacarbazine-based-chemotherapy regimen. Recently antiangiogenic therapy has been reported to provide benefit in MPP patients. Protocols are now available for MPP patients, like the first randomized trial in maligant progressive pheochromocytomas or paragangliomas (FIRSMAPPP) trials in Europe, and can be considered as a first line option in progressive MPP.