Petrowsky Henrik, Schuster Horst, Irani Sarosh, Schäfer Markus, Jochum Wolfram, Schmid Christoph, Boehler Annette, Clavien Pierre-Alain
Swiss Hepato-Pancreato-Biliary Center, Department of Visceral and Transplantation Surgery, University Hospital of Zurich, Switzerland.
Pancreas. 2006 Nov;33(4):430-2. doi: 10.1097/01.mpa.0000236724.49543.f3.
Cystic fibrosis (CF) is the most common lethal genetic disease affecting 1 in 2500 white patients. Chronic obstructive lung disease and pancreatic insufficiency are the main clinical manifestations of CF. Lung transplantation has become a treatment option for advanced pulmonary disease during the last decade. On the other hand, there is evidence from large cohort studies that CF and immunosuppression are risk factors for pancreatic cancer. Here, we report the case of an 18-year-old female patient with CF and bilateral lung transplantation who underwent Whipple procedure for pancreatic adenocarcinoma at the age of 12 years. Because of growth retardation, the patient underwent a 2-year period of growth hormone treatment before the diagnosis of pancreatic cancer. This case should sensitize physicians to be aware of the increased risk for pancreatic cancer in CF patients especially in those after lung transplantation with immunosuppression and prolonged survival.
囊性纤维化(CF)是最常见的致死性遗传疾病,每2500名白人患者中就有1人患病。慢性阻塞性肺疾病和胰腺功能不全是CF的主要临床表现。在过去十年中,肺移植已成为晚期肺部疾病的一种治疗选择。另一方面,大型队列研究的证据表明,CF和免疫抑制是胰腺癌的危险因素。在此,我们报告一例18岁患有CF并接受双侧肺移植的女性患者,该患者在12岁时因胰腺腺癌接受了惠普尔手术。由于生长发育迟缓,该患者在胰腺癌诊断之前接受了为期2年的生长激素治疗。该病例应使医生意识到CF患者尤其是肺移植后接受免疫抑制且生存时间延长的患者患胰腺癌的风险增加。