New Developments in VHL-Associated Neuroendocrine Neoplasms.

IF 4.7 2区医学 Q1 ONCOLOGY Current Oncology Reports Pub Date : 2025-01-01 Epub Date: 2025-01-05 DOI:10.1007/s11912-024-01631-5

Marina Tsoli, Maria Panagaki, Elisavet Tasouli, Dionysia Kolomodi, Gregory Kaltsas

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Abstract

Purpose of review: The purpose of this review is to outline the current knowledge on epidemiology, diagnosis and management of neuroendocrine neoplasms (NENs) that develop in the context of Von Hippel-Lindau (VHL) syndrome.

Recent findings: Pancreatic NENs develop in 8-17% of VHL patients (vPNENs) and are mostly multi-focal, cystic and non-functioning. Surgical resection is recommended for vPNENS > 3 cm that exhibit higher metastatic potential or in tumors with short doubling time while in the 20% of cases with metastatic disease the HIF-2 A inhibitor belzutifan is considered a promising option. Pheochromocytomas arising in VHL type 2 are often bilateral and have a noradrenergic phenotype while they are associated with increased risk of recurrence. High-specific activity [¹³¹I]-MIBG and sunitinib are the treatment options with the highest level of evidence whereas studies on belzutifan are evolving. Life-long surveillance and management in the context of a multidisciplinary team are suggested to achieve the best clinical outcome.

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vhl相关神经内分泌肿瘤的新进展。

综述目的：本综述的目的是概述在Von Hippel-Lindau （VHL）综合征背景下发展的神经内分泌肿瘤（NENs）的流行病学，诊断和治疗的最新知识。近期发现：8-17%的VHL患者（vPNENs）会发生胰腺NENs，多数为多灶性、囊性和无功能。对于具有较高转移潜力或倍增时间短的vPNENS，推荐手术切除，而在20%的转移性疾病病例中，hif - 2a抑制剂贝祖替芬被认为是一个有希望的选择。VHL 2型中出现的嗜铬细胞瘤通常是双侧的，具有去肾上腺素能表型，同时它们与复发风险增加有关。高特异性活性[131I]-MIBG和舒尼替尼是证据水平最高的治疗方案，而对贝祖替芬的研究仍在不断发展。建议在多学科团队的背景下进行终身监测和管理，以达到最佳的临床效果。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Current Oncology Reports ONCOLOGY-

CiteScore

8.50

自引率

0.00%

发文量

187

审稿时长

6-12 weeks

期刊介绍： This journal aims to review the most important, recently published clinical findings in the field of oncology. By providing clear, insightful, balanced contributions by international experts, the journal intends to serve all those involved in the care of those affected by cancer. We accomplish this aim by appointing international authorities to serve as Section Editors in key subject areas, such as cancer prevention, leukemia, melanoma, neuro-oncology, and palliative medicine. Section Editors, in turn, select topics for which leading experts contribute comprehensive review articles that emphasize new developments and recently published papers of major importance, highlighted by annotated reference lists. An international Editorial Board reviews the annual table of contents, suggests articles of special interest to their country/region, and ensures that topics are current and include emerging research. Commentaries from well-known figures in the field are also provided.