Pancreatic cysts are frequently observed in patients with von Hippel-Lindau disease (VHL), and they are considered clinically not important. This study aimed to evaluate the association between pancreatic cysts and diabetes mellitus (DM) in patients with VHL.

Among patients who were on a patient list at the VHL Center at Kyoto University Hospital as of December 2022, those who had undergone an upper abdominal magnetic resonance imaging study after 2010 were retrospectively evaluated. The presence or absence of DM and high glycated hemoglobin (HbA1c) levels (>6.0%) were assessed. Patients were divided into two groups: those with DM or high HbA1c levels, and those without DM or high HbA1c levels. The area of the whole pancreas, including the pancreatic cysts and tumors, the area of the pancreatic cysts, and the percentage of pancreatic cysts, calculated by dividing the area of pancreatic cysts by the area of the whole pancreas, were measured on T2-weighted magnetic resonance images and compared between the two groups.

Thirty-six patients with VHL, comprising 22 men and 14 women, with a mean age of 36.4 years (range, 11-79 years), were identified. Seven patients had DM, and two additional patients had high HbA1c levels. The area of the pancreatic cysts (p = 0.0013) was significantly larger and the percentage of the pancreatic cysts (p = 0.0016) was significantly higher in patients with DM or high HbA1c levels (n = 9) than in patients without DM or high HbA1c levels (n = 27); however, the difference in the area of the whole pancreas was not significant (p = 0.068).

Our findings suggest that patients with VHL who have a large area covered by pancreatic cysts are more likely to have DM than those without.

Pancreatic cystic neoplasms (PCNs) are a diverse group of neoplasms in the pancreas, and are more increasingly encountered with widespread abdominal screening and improved imaging techniques. The most common types of PCNs are serous cystic neoplasms (SCNs), mucinous cystic neoplasms (MCNs), and intraductal papillary mucinous neoplasms (IPMNs). Clinicians frequently feel bewildered in the differential diagnosis and subsequent management among the various types of lesions in the pancreas, which may lead to overtreatment or delayed treatment. The current review provides recent developments in the understanding of the three most common types of PCNs, the latest modalities used in preoperative diagnosis and differential diagnosis, as well as the most up to date management. Suggestions for diagnosis and differential diagnosis of SCNs, MCNs, and IPMNs are also provided for young surgeons. Better understanding of these neoplasms is essential for clinicians to make accurate diagnosis and to provide the best management for patients.