Natural History and Management of Von Hippel-Lindau (VHL) Associated Pancreatic Neuroendocrine Tumors

Launched by NATIONAL CANCER INSTITUTE (NCI) · Aug 28, 2019

Keywords

ClinConnect Summary

This clinical trial is studying how von Hippel-Lindau (VHL) disease affects the pancreas, particularly looking at tumors that can form there. The researchers want to see if a special type of scan, called a 68-Gallium DOTATATE PET/CT, can help detect these tumors earlier and more accurately. By understanding these pancreatic tumors better, the hope is to improve future treatments and care for people with VHL.

To participate in this trial, individuals must be at least 12 years old and have been diagnosed with VHL, showing signs like cysts or tumors in the pancreas. Participants will undergo a series of tests, including physical exams, blood and urine tests, and various imaging scans to monitor their condition. After the initial visit, they will return for follow-up visits every year or every two years for life, depending on their health status. This trial is currently recruiting participants, and it offers an opportunity to contribute to important research that could benefit others with VHL.

Gender

ALL

Eligibility criteria

• * INCLUSION CRITIERIA:
• 1. Participants who have been diagnosed with VHL using the following criteria:
• • -- Identification of a heterozygous germline pathogenic variant in VHL by molecular genetic testing.
• • or
• • -- Clinical criteria
• 2. Participants with at least 1 pancreatic manifestation of VHL as documented on any non-invasive imaging study. These manifestations may include:
• • Pancreatic cyst(s)
• • Solid lesions suspicious for microcystic adenoma(s)
• • Solid enhancing lesions suspicious for PNET(s)
• • Any other solid lesion(s) of the pancreas
• • 3. Age greater than or equal to 12 years.
• • 4. Ability of participant to understand and the willingness to sign a written informed consent document.
• EXCLUSION CRITERIA:
• • 1. Inability of participant to undergo serial non-invasive imaging.