Risk stratification for surgical management of patients with von Hippel-Lindau (VHL) disease with pancreatic solid lesions on anatomical imaging^[1,2]

VHL: von Hippel-Lindau; pNET: pancreatic neuroendocrine tumor.
* Surgical resection may be an option in select patients with limited, potentially resectable oligometastatic disease. Systemic therapy options are similar to those used for sporadic pNETs. The use of belzutifan in this setting requires further investigation. Refer to UpToDate content on systemic therapy for metastatic pNETs.
¶ Pancreas-preserving surgery is emphasized in patients with VHL-associated pNET, given tumor multifocality and the potential need for future pancreatic resections. Surgical principles are otherwise similar to those of sporadic pNET. Refer to UpToDate content on surgical resection of sporadic pNETs.
Δ Patients with a missense or exon 3 pathogenic or likely pathogenic variant are at higher risk for metastatic disease and requiring surgical intervention^[3,4]. Refer to UpToDate content on VHL and pancreatic tumors.
◊ Belzutifan is an alternative to imaging surveillance for patients with rapidly growing tumors (ie, accelerated doubling time <500 days) or those with resectable disease who wish to potentially delay or defer surgical intervention.