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Related Experiment Video

Updated: Jun 3, 2025

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New Developments in VHL-Associated Neuroendocrine Neoplasms.

Marina Tsoli1, Maria Panagaki2, Elisavet Tasouli2

  • 1Neuroendocrine Tumour Unit, ENETS Centre of Excellence, 1st Department of Propaedeutic and Internal Medicine, Laiko Hospital, National and Kapodistrian University of Athens, Agiou Thoma 17, Athens, 11527, Greece. martso.mt@gmail.com.

Current Oncology Reports
|January 5, 2025
PubMed
Summary

Von Hippel-Lindau (VHL) syndrome can cause neuroendocrine neoplasms (NENs), particularly pancreatic NENs and pheochromocytomas. Management involves surveillance, surgery for larger tumors, and emerging therapies like belzutifan.

Keywords:
BelzutifanPancreatic neuroendocrine neoplasmsPheochromocytomaVHL

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Area of Science:

  • Neuroendocrinology
  • Oncology
  • Genetics

Background:

  • Von Hippel-Lindau (VHL) syndrome is a genetic disorder predisposing individuals to various tumors.
  • Neuroendocrine neoplasms (NENs) are a significant concern in VHL patients.

Purpose of the Study:

  • To review the current understanding of epidemiology, diagnosis, and management of NENs in VHL syndrome.
  • To consolidate knowledge on pancreatic NENs (vPNENs) and pheochromocytomas in VHL patients.

Main Methods:

  • Literature review of epidemiological data, diagnostic criteria, and therapeutic strategies for VHL-associated NENs.
  • Analysis of current treatment guidelines and emerging therapeutic options.

Main Results:

  • Pancreatic NENs (vPNENs) occur in 8-17% of VHL patients, often multifocal and non-functioning; surgical resection is indicated for tumors >3 cm or with rapid growth.
  • Pheochromocytomas in VHL type 2 are frequently bilateral, noradrenergic, and recurrent; [131I]-MIBG and sunitinib are established treatments, with belzutifan showing promise.
  • Belzutifan, a HIF-2α inhibitor, is a potential treatment for metastatic disease in VHL-associated NENs.

Conclusions:

  • Lifelong surveillance and multidisciplinary management are crucial for optimal outcomes in VHL patients with NENs.
  • Early detection and tailored treatment strategies are essential for managing vPNENs and pheochromocytomas.
  • Emerging therapies like belzutifan offer new hope for managing advanced or metastatic VHL-associated NENs.