Pancreatic involvement in Von Hippel-Lindau disease: the role of integrated imaging.

Von Hippel-Lindau disease is a rare dominant autosomal genetic disease determined by the inactivity of an onco-suppressor gene identified in 1993 and located on the short arm of chromosome 3 [1]. This gene has a role of crucial importance in the regulation of the vascular endothelial growth factor (VEGF); therefore, its inactivation promotes an overexpression of the VEGF and the angiogenesis of the tumor characterizing this syndrome [2]. The main clinical and radiological manifestations of the disease are hemangioblastoma of the retina and of the nervous central system, clear cell renal tumors, pheochromocytoma, cystadenoma of the epididymis, tumors of the endolymphatic sack, carcinoid tumors, pancreatic tumors, and multiple cystic formations of the pancreas as well as of the kidneys and the epididymis [2, 3]. Pancreatic gland involvement ranges from 15 to 77% of the cases [1]. An ultrasound scan (US), computed tomography (CT), and magnetic resonance imaging (MRI) play a crucial role both in the initial evaluation of the various manifestations of the disease as well as in the follow-up of these patients [4]. We herein show an integrated imaging study using CT and MRI in evaluating the involvement of the pancreatic gland in Von Hippel-Lindau syndrome. A 58-year-old female patient, suffering from Von Hippel-Lindau syndrome diagnosed ten years earlier, without any symptom of possible pancreatic localization was admitted to our hospital because of decompensated type 2 diabetes. When she was 47-year-old, she underwent surgery for a cerebellar hemangioblastoma, the first illness manifestation. Subsequently, the patient underwent an imaging follow-up with US and CT. Eight years later, due to the presence of two renal lesions at US, the patient underwent a CT scan. This examination confirmed the presence of two renal lesions, with a partially exophytic development, hypodense at examination without contrast medium but showing a meaningful enhancement after the administration of the iopamidol (Iopamiro, Bracco S.p.A., Milan, Italy). One lesion (the larger having a diameter of 4 cm) was located in the middle part of the right kidney and the smaller one in the upper part of the left