The invited discussant of the FIRSTMAPP study, Rocio Garcia-Carbonero, MD, of Hospital Universitario 12 de Octubre, Madrid, emphasized that pheochromocytoma and paraganglioma are rare tumors, and once they become metastatic, which is even more rare, there are few treatment options.
“This is very challenging from a clinical point of view. These tumors have a wide distribution anatomically. There are no robust predictors of metastasis. Once we are facing a metastatic disease, our treatment options are limited,” she emphasized. They include metaiodobenzylguanidine combined with radioactive iodine, peptide receptor radionuclide therapy, or chemotherapy.
Dr. Garcia-Carbonero was enthusiastic about these groundbreaking results. Currently, tyrosine kinase inhibitors are not included in clinical recommendations from the National Comprehensive Cancer Network or European guidelines. “There is only poor evidence for other treatment options,” she noted.
“The efficacy of sunitinib is particularly relevant, but not restricted to SDHB-mutated tumors. Safety seems reasonable and manageable,” Dr. Garcia-Carbonero said.
Rocio Garcia-Carbonero, MD
“FIRSTMAPP is a positive trial. Sunitinib is active in these patients. This is the first randomized trial and largest trial ever conducted in the field of metastatic pheochromocytoma and paraganglioma. This is the highest level of evidence ever reached in this very rare cancer,” she said.
“The efficacy reported [with sunitinib] is in the range of other systemic treatment options included in current clinical guidelines. I agree with Dr. Baudin—this is practice-changing, and sunitinib has become the therapeutic option with the most solid and robust antitumor activity that we have to date,” Dr. Garcia-Carbonero concluded.
DISCLOSURE: Dr. Garcia-Carbonero has received honoraria from AAA, Advanz Pharma, Amgen, Bayer, Bristol Myers Squibb, Harvest Moon Pharmaceuticals, Ipsen, Lilly, Merck, Midatech Pharma, Merck Sharp & Dohme, Novartis, PharmaMar, Pfizer, Pierre Fabre, Roche, Servier, and Sanofi; and has received research support from Pfizer, Bristol Myers Squibb, and Merck Sharp & Dohme.
Results from the phase II FIRSTMAPPP study support the potential use of the tyrosine kinase inhibitor sunitinib in two rare neuroendocrine malignancies—pheochromocytoma and paraganglioma. Sunitinib significantly improved the primary endpoint of progression-free survival vs placebo, according to...