Related Experiment Video
Updated: Feb 23, 2026

A Patient-Derived Xenograft Model for Venous Malformation
Published on: June 15, 2020
[Gastroenterological manifestations of von Hippel-Lindau disease]
Joanna Koniusz1, Krzysztof Dąbkowski2, Katarzyna Buczek2
1Department of Gastroenterology, Pomeranian Medical University, Szczecin, Poland; Department of General, Vascular and Transplantation Surgery, Marie Curie Hospital, Szczecin, Poland.
Abstract:
Von Hippel-Lindau disease is rare autosomal dominant disorder that results from mutation of VHL gene. Typical manifestations of this syndrome include haemangioblastomas of retina, cerebellum and spinal cord, endolymphatic sac tumors, clear cell cancer and kidney cysts, pheochromocytoma, pancreatic cysts and neuroendocrine tumors. The differential diagnosis of pancreatic lesions in patients with von Hippel Lindau syndrome plays an important role. The pancreas in VHL disease is not only site of benign lesions (cysts, serous systic adenomas) but also of potentially malignant (neuroendocrine) and malignant tumors(metastases).The gastroenterological manifestations can be the first symptoms of von Hippel-Lindau disease.
Related Concept Videos
Endocarditis II: Clinical Features of Infective Endocarditis
Chronic Kidney Disease II: Clinical Manifestations
Peptic Ulcer Disease III: Clinical Manifestations and Diagnostic Studies
Few clinical manifestations differentiate gastric ulcers from duodenal ulcers. Distinctions in the location, timing, and pain relief are crucial for healthcare providers in differentiating between gastric and duodenal ulcers during clinical assessments.
Esophageal Varices-II: Clinical Features and Management
In the initial assessment, a thorough review of the patient's medical history is vital to identify risk factors such as liver disease, alcohol...
Notch Signaling Pathway
The Notch gene came into the limelight in 1914 after the discovery that its mutation in Drosophila melanogaster leads to a serrated (or "notched") wing margin phenotype. It was not...
Peripheral Arterial Disease II: Clinical Manifestations and Diagnostic Evaluation

