Endocrine Abstracts

Background: Sympathetic pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors associated with excess catecholamine production. Routine biochemical and imaging surveillance to monitor for recurrence and metastasis is recommended. However, there is limited data describing optimal surveillance approaches and post-operative biochemical thresholds for detecting recurrences or metastases. This study sought to correlate biochemical and imaging surveillance with ...

Background: Patients with advanced pheochromocytoma/paraganglioma (PPGL) or pancreatic neuroendocrine tumor (panNET) are in need of novel targeted therapies. Hypoxia-inducible factor 2α (HIF-2α) is one of the key oncogenic drivers in neuroendocrine tumors. Hypoxia signaling pathway alterations or other mechanisms that stabilize HIFs are common in some PPGLs and panNETs. Belzutifan (MK-6482), a HIF-2α inhibitor, has shown antitumor activity in advanced renal cell...

Background: Metastatic pheochromocytomas and paragangliomas are orphan neuroendocrine tumors. 50% of these tumors are associated with germline mutations of the SDHB gene. SDHB related pheochromocytomas and paragangliomas and many apparently sporadic tumors exhibit abnormal angiogenesis. This trial assessed the efficacy and safety of Cabozantinib, a potent, antiangiogenic, tyrosine kinase inhibitor. Methods: The Natalie trial is a single arm phase 2 clini...

Background: Metastatic pheochromocytomas and paragangliomas are rare neuroendocrine tumors with limited treatment options. We studied the efficacy and safety of off-label High-Specific-Activity I-131-meta-iodobenzylguanidine (HSA-I-131-MIBG) in routine clinical practice. Methods: This is a retrospective cohort study. The primary endpoint is objective response rate (ORR) as per RECIST v1.1. Secondary endpoints are blood pressure control, safety, overall a...