Primary Gallbladder Carcinoma in a Pregnant Patient with Crohnʼs Disease Complicated with Gallbladder Involvement

Abstract

Purpose: Primary gallbladder (GB) carcinoma and Crohn's disease (CD) of the GB are individually rare. We present a case of a pregnant woman with CD found to have GB involvement and primary GB carcinoma. Case: A 34-year-old female at 6 weeks gestation with a 21 year history of CD of uncertain extent presented with 3 months of diarrhea, urgency, and abdominal pain. Her disease had been indolent for the majority of her life. She was adherent to her medications, mesalamine and mercaptopurine, as needed at times of symptoms. Sigmoidoscopy showed mild patchy colitis of the descending and sigmoid colon with rectal sparing. Pathology showed moderately active chronic colitis with crypt distortion and cryptitis without granuloma. She was started on oral and topical mesalamine. Initial labs included abnormal alkaline phosphatase 515 U/L, AST 96 U/L, and ALT 181 U/L. Further labs included a negative anti-mitochondrial antibody (AB), anti-smooth muscle AB, celiac and viral hepatitis serologies and anti-nuclear AB. A non-contrast MRCP was obtained revealing two hypointense smooth margined masses with small stalks in the neck and fundus of the GB, 1.4 × 1.8 cm and 1.4 × 2.3 cm, respectively. No GB wall thickening, cholilithiasis, ductal dilation, strictures, or liver parenchymal abnormalities were present. Laparoscopic cholecystecomy was performed at 18 wks gestation. Histology revealed a poorly differentiated adenocarcinoma of the GB with invasion through the muscularis propria and an adjacent tubulovillous adenoma with highgrade dysplasia without nodal involvement (stage pT2N0). Additionally, there was widespread epithelial dysplasia in the gallbladder with acute superficial inflammation, transmural chronic inflammation with numerous plasma cells and one granuloma consistent with CD. Muscularis propria invasion prompted partial liver resection and portal lymphadenectomy, revealing matted lymph nodes in the porta hepatis positive for metastatic carcinoma (stage pT2N1). Liver tissue was negative for primary sclerosing cholangitis. Conclusion: Seven cases of CD involving the GB and 3 cases of primary GB carcinoma in CD have been published. This is the only case that describes both. Common features in CD of the GB include acute cholecystitis, ileal involvement, and presence independent of active intestinal disease. Common features in CD patients with GB malignancy include younger age of detection, a long history of CD, extensive colonic and ileal involvement, and absence of cholelithiasis. Adnomatous GB polyps and pregnancy is specific to this case. The role of CD in the development of GB malignancy is not well understood; however, longstanding immunosuppression is implicated.