Sousa Ana Lúcia, Sousa Diamantino, Figueiredo Pedro, Marques Pedro Pinto, Guerreiro Horácio
Ana Lúcia Sousa, Diamantino Sousa, Horácio Guerreiro, Department of Gastroenterology, Hospital de Faro E.P.E., 8000-386 Faro, Portugal.
World J Gastrointest Endosc. 2013 Oct 16;5(10):519-22. doi: 10.4253/wjge.v5.i10.519.
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.
在此，我们报告一例35岁女性患者，长期患有消化不良，影像学检查显示胰腺头部和尾部存在多个囊肿。患者接受了超声内镜检查，证实整个胰腺存在多个单纯囊肿，胰管未扩张。大多数囊肿大小小于1厘米，最大的囊肿呈蜂窝状外观。对两个最大囊肿的抽吸物进行细胞学检查，结果与良性胰腺囊肿相符。超声内镜还显示左肾和脾脏内有囊肿。基因检测确诊为冯·希佩尔-林道病。我们强调这个病例，因为对于冯·希佩尔-林道病这种罕见的临床病症来说，仅表现为囊肿而无中枢神经系统血管母细胞瘤和恶性肿瘤等更常见表现是不寻常的。
