Published online Oct 16, 2013. doi: 10.4253/wjge.v5.i10.519
Revised: August 25, 2013
Accepted: September 4, 2013
Published online: October 16, 2013
Here we present the case of a 35-year-old female patient with long standing dyspepsia and imaging studies showing the presence of multiple cysts in the head and tail of the pancreas. The patient underwent endosonography that confirmed the presence of multiple simple cysts throughout the entirety of the pancreas without dilation of the pancreatic duct. The majority of the cysts were less than one centimeter in size, and the largest cyst showed a honeycomb appearance. Cytology of aspirates from the two largest cysts was compatible with benign pancreatic cysts. Endosonography also revealed cysts within the left kidney and spleen. Genetic testing confirmed Von Hippel-Lindau disease. We highlight this case because it is unusual for Von Hippel-Lindau disease, a rare clinical entity, to present solely with cysts in the absence of more common manifestations, such as hemangioblastomas in the central nervous system and malignancy.
Core tip: This is a case of a rare clinical entity, Von Hippel-Lindau disease, with an unusual presentation. The patient had only pancreatic cysts without more common manifestations, particularly hemangioblastomas and malignancy. The imaging methods used in this case were important for the diagnosis, particularly endosonography, which showed the honeycomb appearance of the pancreatic serous cystadenomas. This case should alert endoscopists to the possible occurrence of this hereditary disease in the presence of multiple pancreatic cysts without other manifestations or family history.