Rapidly progressive metastatic cholangiocarcinoma in a postpartum patient with cystic fibrosis: a case report
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CASE REPORT
Open Access
Rapidly progressive metastatic cholangiocarcinoma in a postpartum patient with cystic fibrosis: a case report Sara W. Carson1* , Kelly E. Craven2, David Nauen2, Kristina Montemayor1, Mark Yarchoan3, William R. Burns4, Christian A. Merlo1,5 and Natalie E. West1
Abstract Background: Cholangiocarcinoma is a rare gastrointestinal malignancy that arises within the intrahepatic, perihilar, and/or extrahepatic bile ducts. Individuals with cystic fibrosis are at increased risk for gastrointestinal malignancies. The most common gastrointestinal malignancy in cystic fibrosis is colon cancer, but other gastrointestinal malignancies also occur at greater rates than the general population. Case presentation: We present a case of a rapidly progressive metastatic intrahepatic cholangiocarcinoma in an individual with cystic fibrosis who was 5 months postpartum, incidentally found while undergoing a lung transplantation evaluation. Conclusion: A heightened clinical awareness of gastrointestinal malignancies, beyond colon cancer, in individuals with cystic fibrosis is warranted. It remains unclear if pregnancy is an additional risk factor for gastrointestinal malignancies in cystic fibrosis. Keywords: Cholangiocarcinoma, Gastrointestinal cancer, Cystic fibrosis, pregnancy, Case report
Background Individuals with cystic fibrosis (CF) are at higher risk for digestive tract cancers [1, 2]. Cystic fibrosis transmembrane conductance regulator (CFTR)-regulated chloride channels are highly expressed in the gastrointestinal tract and when disrupted, as in CF, there is increased inflammation and cell turnover, which is believed to contribute to this increased risk [3, 4]. Furthermore, there is limited evidence that pregnancy may decrease the maternal immune system and thus increase the risk of, or at least increase the progression of malignancy [5–8]. However, it is unknown if pregnancy itself would have an additive effect on the increased risk of digestive tract * Correspondence: [email protected] 1 Department of Medicine, Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, 1830 E. Monument St 5th Floor, Baltimore, MD 21205, USA Full list of author information is available at the end of the article
cancers in women with CF. We report a case of a woman with CF who was 5 months postpartum who was diagnosed with rapidly progressive metastatic intrahepatic cholangiocarcinoma incidentally discovered during evaluation for lung transplantation.
Case presentation A 26-year-old female with CF (genotype F508del/ F508del), with a baseline forced expiratory volume in 1 s (FEV1) of 21% predicted and a 6-l home oxygen requirement, was undergoing evaluation for bilateral lung transplantation. Her medical history included recurrent respiratory infections with multi-drug resistant pathogens, pancreatic insufficiency, malnutrition requiring percutaneous gastrostomy feeds, atrioventricular nodal reentry tachycardia status post ablation, and the recent delivery of a healthy baby. She had no history of bilia
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