RT Journal Article T1 Successful Second-Line Metronomic Temozolomide in Metastatic Paraganglioma: Case Reports and Review of the Literature. A1 Tena, Isabel A1 Gupta, Garima A1 Tajahuerce, Marcos A1 Benavent, Marta A1 Cifrián, Manuel A1 Falcon, Alejandro A1 Fonfria, María A1 Del Olmo, Maribel A1 Reboll, Rosa A1 Conde, Antonio A1 Moreno, Francisca A1 Balaguer, Julia A1 Cañete, Adela A1 Palasí, Rosana A1 Bello, Pilar A1 Marco, Alfredo A1 Ponce, José Luis A1 Merino, Juan Francisco A1 Llombart, Antonio A1 Sanchez, Alfredo A1 Pacak, Karel K1 Paraganglioma K1 SDHB K1 metastatic K1 metronomic K1 temozolomide AB Metastatic pheochromocytoma and paraganglioma (mPHEO/PGL) are frequently associated with succinate dehydrogenase B (SDHB) mutations. Cyclophosphamide-dacarbazine-vincristine (CVD) regimen is recommended as standard chemotherapy for advanced mPHEO/PGL. There is limited evidence to support the role of metronomic schemes (MS) of chemotherapy in mPHEO/PGL treatment. We report 2 patients with SDHB-related mPGL who received a regimen consisting of MS temozolomide (TMZ) and high-dose lanreotide after progression on both CVD chemotherapy and high-dose lanreotide. Molecular profiling of the tumor tissue from both patients revealed hypermethylation of the O6-methylguanine-DNA-methyltransferase (MGMT) promoter. In one patient, progression-free survival was 13 months and the second patient remained under treatment after 27 months of stabilization of metabolic response of his disease. Treatment was well tolerated, and adverse effects were virtually absent. A modification in the scheme of TMZ from standard schemes to MS is safe and feasible and can be considered in patients with progressive mPHEO/PGL refractory to dacarbazine in standard doses. SN 1179-5549 YR 2018 FD 2018-04-09 LK https://hdl.handle.net/10668/28158 UL https://hdl.handle.net/10668/28158 LA en DS RISalud RD Apr 17, 2025