Functional profiling of somatostatin receptors identifies somatostatin receptor subtype 2 as a vulnerability in Succinate Dehydrogenase SDHB-deficient pheochromocytomas and paragangliomas
Víctor García-Vioque·Alejandro Ibañez-Costa·María Trinidad Moreno-Montilla·Esther Rivero-Cortés·Ricardo Blazquez-Encinas·Federica Mangili·Ester Arroba·Aura Dulceida Herrera-Martinez·Michael D. Culler·María Ángeles Gálvez-Moreno·Anne Barlier·Luisa M. Botella·Mercedes Robledo·Justo P. Castaño·Sergio Pedraza-Arevalo
Abstract Pheochromocytomas and Paragangliomas (PPGL) are rare neuroendocrine tumors with favorable prognosis, although a significant subset (20–25%) progress to metastasis, worsening patient prognosis. For metastatic cases, pharmacological interventions become essential, yet most tumors show poor response to treatment. While clinical trials are ongoing, there is no established treatment for metastatic PPGL. Like other neuroendocrine tumors, PPGL exhibit high membrane expression of somatostatin r