Endocrine Abstracts

Introduction: VIPomas are rare neuroendocrine tumors (NET), usually located in the pancreas that secrete vasoactive intestinal polypeptide (VIP) leading to high-volume watery diarrhea and complications such as hypokalemia, acidosis and dehydration. The management of VIPoma symptoms is often challenging when surgical resection of all the lesions is not feasible. Treatments are then based on somatostatin analogs (SSA) and may also include hepatic chemoembolization, systemic chem...

Introduction: Malignant paraganglioma/pheochromocytoma (MPP) are very rare neuroendocrine tumors with heterogeneous prognostic and no gold-standard treatment. MPP can be associated with germline mutations at SDH-x genes which encode for the succinate dehydrogenase that catalyzes the oxidation of succinate to fumarate. SDH-x mutations lead to inactivation of the enzyme and thus accumulation of succinate.Aim: This project aims to evaluate...

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...

Interferon-α (IFN-α) has shown some activity in neuroendocrine tumors with disease stabilizations. Malignant pheochromocytoma and paraganglioma (MPPGLs) have a heterogeneous behavior with a slow progression rate, most of the time and a high frequency of bone metastases. Stabilizing disease and preventing skeletal-related events are two goals to achieve in the management of MPPGLs patients.This retrospective study evaluated a multimodal strategy...

Purpose: Bone metastases (BM) are frequent in differentiated thyroid cancer (DTC). These patients may present skeletal-related events (SRE), the leading cause of DTC-related morbidity. We aimed to evaluate the clinical features, treatment approaches, and outcomes including overall survival (OS) of DTC patients with BM complicated by SRE and their evolution over time.Methods: 178 consecutive DTC patients harbouring BM were enrolled in this retrospective s...

Objectives: RET alterations are targetable oncogenic drivers in TC. Patients with TC, especially those with MTC treated with the multikinase inhibitors cabozantinib and/or vandetanib (C/V), often experience significant treatment-related side effects. Pralsetinib, a selective RET tyrosine kinase inhibitor, showed efficacy in patients with RET-altered TC from the phase 1/2 ARROW trial (NCT03037385). We present the impact of pralsetinib on PROs in patients with ...

Background: Presence of venous vascular invasion is a criterion of intermediate risk of recurrence in papillary thyroid carcinoma (PTC). However, the presence and type of vascular invasion (lymphatic or venous) is often underreported and its impact on PTCs without other risk features remains unknown.Objective: To evaluate the impact of both lymphatic and venous invasion on the risk of recurrence/persistence on otherwise low-risk PTCs. <p class="abste...

Background: Presence of venous vascular invasion is a criterion of intermediate risk of recurrence in papillary thyroid carcinoma (PTC). However, the presence and type of vascular invasion (lymphatic or venous) is often underreported and its impact on PTCs without other risk features remains unknown.Objective: To evaluate the impact of both lymphatic and venous invasion on the risk of recurrence/persistence on otherwise low-risk PTCs.<p class="abstex...

Context: Paragangliomas and pheochromocytomas (PPGL) are rare neuroendocrine tumors, characterized by a strong genetic component. Indeed, up to 40% of patients carry a germline mutation in a PPGL susceptibility gene. In accordance with the international recommendations, genotyping of PPGL susceptibility genes is therefore proposed to all patients with PPGL, but it has actually never been shown whether the identification of a germline mutation in one PPGL susceptibility gene ch...