Endocrine Abstracts

Background: Gastroenteropancreatic Neuroendocrine Neoplasms (GEP-NENs) are a rare subset of cancers which nevertheless are a rising health burden. Development of new therapies suffers from several bottlenecks, including low patient accrual and poor understanding of tumor characteristics. Patient tumor organoids (PTOs) are a novel model capable of improving screening of patient tissue in an accurate, standardized, and high-throughput capacity. In this study, we utilized patient...

Background: Patients with neuroendocrine tumors (NETs) are at risk of carcinoid crisis when undergoing operations, which is associated with adverse post-operative outcomes. There is no contemporary guideline regarding the perioperative management of patients at risk of carcinoid crisis. Moreover, recent evidence has challenged traditional management with somatostatin analogs preparation and avoidance of beta-adrenergic medication. Therefore, specific guidance is needed to prov...

Background: Neuroendocrine tumors (NETs) are a heterogeneous group of malignant neoplasms arising from neuroendocrine cells distributed throughout the body. The most common sites of NETs are the gastrointestinal tract, pancreas and lungs. The clinical management of NETs is not standardized, with few FDA-approved therapies. Moreover, drug development has been challenging for NETs due to limited pre-clinical models. To address this unmet need, the NCI Natural History Study of Ch...

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: GI neuroendocrine tumors express somatostatin (SSTR) receptors, and the radioloabeled SSTR analog Lu-177-DOTATATE is an FDA-approved systemic treatment for those with metastatic disease. Olaparib is a poly (ADP-ribose) polymerase inhibitor (PARPi) which inhibits cells from repairing damaged DNA, especially single-stranded DNA breaks caused by beta particle emissions from the radioactive decay of Lu-177. We report the first results of a phase 1/2 study evaluating th...

Background: Metastatic GI Neuroendocrine Tumors (GI-NET) and pheochromocytoma/paraganglioma (PPGL) are are tumors which overexpresses somatostatin receptors (SSTR) and can be treated with targeted radioligand therapy (RLT) such as Lu-177-DOTATATE. However, despite demonstrated clinical efficacy at stabilizing tumor growth, durable response is very rare and almost all patients inevitably progresses at some time after treatment. Good systemic therapy options after beta-emiting R...

Background: Pheochromocytoma/Paraganglioma (PPGL) are rare neuroendocrine tumors that expresses somatostatin receptors (SSTR) and can be treated with radiolabeled somatostatin analogues such as Lu-177-DOTATATE. Ga-68-DOTATATE PET scans show the distribution and density of SSTR+ tumors, and F-18-FDG PET scans show hypermetabolism in lesions. Total uptake volumes (TUV) for each respective scan can be obtained consistently and semi-automatically using standardized workflow in ima...

Background: Pheochromocytoma/Paraganglioma (PPGL) are rare neuroendocrine tumors that expresses somatostatin receptors (SSTR) and can be treated with radiolabeled somatostatin analogues such as Lu-177-DOTATATE. This study is the first prospective study to examine the safety and efficacy of Lu-177-DOTATATE in the treatment of metastatic or inoperable PPGL in a phase 2 clinical trial setting, and results from a planned interim analysis are presented.Method...

Background: Somatostatin receptors (SSTR) is overexpressed in a number of different tumors, including GI Neuroendocrine Tumors (GI-NET), Pheochromocytoma/Paraganglioma (PPGL), small cell lung cancer (SCLC), renal cell carcinoma (RCC), and certain head and neck cancers (H&N) such as olfactory neuroblastoma. It has been demonstrated that these SSTR-expressing tumors can be treated with beta-emitting radioligand therapy (RLT) that binds to SSTR such as Lu-177-DOTATATE. Howeve...