Department of Internal Medicine and Gastroenterology, Pancreatic Unit, Fondazione Policlinico Universitario "A. Gemelli", Catholic University of the Sacred Heart, School of Medicine, Rome, Italy.
Eur Rev Med Pharmacol Sci. 2017 Nov;21(22):5179-5184. doi: 10.26355/eurrev_201711_13837.
Pancreatic cystosis is a rare presentation of cystic fibrosis involving pancreatic gland. To date, only very few cases of pancreatic cystosis have been described in literature. Pancreatic cystosis may begin during the second decade of life and is the rarest presentation of cystic fibrosis. This disease is characterized by the presence of multiloculated cysts without ductal system communication of different sizes in all the pancreatic tissue. Herein, we report a case of a young woman affected by cystic fibrosis that was admitted to our Pancreatic Centre to evaluate a picture of diffuse multiloculated pancreatic cysts. After magnetic resonance imaging (MRI) and endoscopic ultrasound (EUS) assessment, we perform the diagnosis of the concomitant presence of the rare condition of pancreatic cystosis with Branch Duct-Intraductal Papillary Mucinous Neoplasm (BD-IPMN). To our knowledge, this is the first reported case of a cystic fibrosis patient with the combination of pancreatic cystosis and IPMN.
胰腺囊性病是囊性纤维化累及胰腺的一种罕见表现。迄今为止,文献中仅描述了极少数胰腺囊性病病例。胰腺囊性病可在生命的第二个十年开始,并是囊性纤维化最罕见的表现。这种疾病的特征是在所有胰腺组织中存在大小不一的多房性囊肿,但没有导管系统的沟通。在此,我们报告一例年轻女性患有囊性纤维化,因弥漫性多房性胰腺囊肿就诊于我院胰腺中心。经磁共振成像(MRI)和内镜超声(EUS)评估后,我们诊断为同时存在罕见的胰腺囊性病和分支胰管-内乳头状黏液性肿瘤(BD-IPMN)。据我们所知,这是首例报道的囊性纤维化患者同时合并胰腺囊性病和 IPMN。