VHL and pancreatic neuroendocrine tumor: For example, pancreatic NETs arising in VHL disease tend to be indolent: a cohort study noted superior long-term survival in patients with VHL after resection compared with matched sporadic cases.114 In contrast, MEN1 usually presents with multifocal PNETs and higher metastatic potential as tumors enlarge.115 Familial context thus informs a tailored approach, ranging from active surveillance of tiny lesions to timely resection in high-risk cases.