Endocrine Abstracts

There is currently no curative treatment for metastatic phaeochromocytoma (PH) and paraganglioma (PG). Patient management aims at reducing tumor burden and preventing mechanical and secretory consequences of metastatic spread and must be adapted to disease progression. Focal treatments (surgery, radiofrequency ablation and embolization) reduce hypersecretion and facilitate subsequent systemic therapies using radionuclides (131I-MIBG, 177Lu- or 90Y-DOTATOC), chemotherapy (cyclophosphamide- and dacarbazine-based regimens combined with vincristine or doxorubicin) and antiangiogenic therapy. Inhibiting the effect of HIF-2 alpha activation-dependent targets has become an attractive therapeutic goal for patients with metastatic PH/PG. Recent publications have reported objective responses with manageable toxicity in patients treated with sunitinib. A randomized, double-blind, phase II, international, multicenter study with sunitinib 37.5 mg daily started by the end of 2011 in Europe (ENS@T network). This study’s primary endpoint is progression-free survival at 12 months.

Declaration of interest: There is no conflict of interest that could be perceived as prejudicing the impartiality of the research reported.

Funding: No specific grant from any funding agency in the public, commercial or not-for-profit sector.