Case Report
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World J Gastrointest Endosc. Oct 16, 2013; 5(10): 519-522
Published online Oct 16, 2013. doi: 10.4253/wjge.v5.i10.519
Contribution of endosonography in an uncommon case of pancreatic cysts
Ana Lúcia Sousa, Diamantino Sousa, Pedro Figueiredo, Pedro Pinto Marques, Horácio Guerreiro
Ana Lúcia Sousa, Diamantino Sousa, Horácio Guerreiro, Department of Gastroenterology, Hospital de Faro E.P.E., 8000-386 Faro, Portugal
Pedro Figueiredo, Pedro Pinto Marques, Department of Gastroenterology, Hospital Garcia de Orta, 2801-951 Almada, Portugal
Author contributions: Sousa AL and Sousa D contributed equally to this work; Sousa AL and Sousa D were attending doctors for the patient and designed and wrote the article; Figueiredo P and Marques PP performed the endosonography and critically revised the article; Guerreiro H approved the final version to be published.
Correspondence to: Ana Lúcia Sousa, MD, Department of Gastroenterology, Hospital de Faro E.P.E., Faro, Rua leão Penedo, 8000-386 Faro, Portugal.
Telephone: +351-91-4234342 Fax: +351-28-9891211
Received: July 12, 2013
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.

Keywords: Von Hippel-Lindau disease, Endosonography, Pancreatic cysts, Hereditary disease, Cysts

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.