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Xanthogranulomatous Cholecystitis as the Cause of Abdominal Pain in a 15-Year-Old Boy
ELECTRONIC IMAGE OF THE MONTH Xanthogranulomatous Cholecystitis as the Cause of Abdominal Pain in a 15-Year-Old Boy Febin John,* Dovid Moradi,‡ and Arkady Broder‡ *Department of Internal Medicine, ‡Department of Gastroenterology, Saint Peters University Hospital, Robert Wood Johnson Medical School, Rutgers University, New Brunswick, New Jersey
15-year-old boy with a history of autism spectrum disorder was admitted to our hospital with abdominal pain. He originally had presented 2 weeks earlier with abdominal pain but was discharged after ruling out appendicitis by an ultrasound. He returned with ongoing pain in the right upper quadrant (RUQ) with postprandial vomiting and fever. Vitals were as follows: blood pressure, 101/57 mm Hg; pulse rate, 104/ min; temperature, 99.1 F; and body mass index, 18.3 kg/ m2. Abdominal examination showed a firm and tender RUQ with negative Murphy’s sign. The patient’s white blood cell count was 15,800 cells/ mm3 with 78.3% neutrophils, hemoglobin level was 11.8 g/ dL, total bilirubin level was 2.9 mg/dL with direct bilirubin of 2.1 mg/dL, aspartate aminotransferase level was 29 U/L, alanine aminotransferase level was 32 U/L, alkaline phosphatase level was 317 U/L, g-glutamyltransferase level was 309 U/L, international normalized ratio was 1.43, and C-reactive protein level was 300 mg/L. All other laboratory results were within normal limits. RUQ ultrasound (Figure A) showed gallstones and a complex cystic area (10.3 6.8 7.8 cm) without internal vascularity, confirmed by a computed tomography scan of the abdomen (Figure A), which showed large multiloculated cystic lesion with septations in the region of the porta hepatis and gallbladder (GB) fossa measuring 8.2 7.5 10.0 cm with mass effect leading to Mirizzi syndrome.
A
The patient was managed with intravenous fluids and antibiotics. Endoscopic retrograde cholangiopancreatography showed a smooth common bile duct stricture with intrahepatic biliary dilation with dye filling the cystic mass (Figure B). Endoscopic ultrasound–guided fine-needle aspiration was negative for malignant cells and hence a frozen section was not performed. Aerobic and anaerobic cultures remained sterile. Subsequently, the patient underwent exploratory laparotomy with complete resection of the large multicystic mass (Figure C). Pathology showed a mass consistent with GB with severe acute and chronic inflammation and multiple granulomas (Figure C). He was discharged home after 16 days of hospitalization and was asymptomatic at his follow-up visit.
Discussion Xanthogranulomatous cholecystitis (XGC) is a rare variant of chronic cholecystitis characterized by destructive inflammation of the GB.1–3 XGC first was described by McCoy et al4 in 1976, representing approximately 10% of all cases of cholecystitis.2 With a slight male preponderance, XGC is seen rarely in young patients.2 The inflammation can be intense, with infiltration and fibrosis leading to asymmetric thickening of the GB wall and formation of multiple nodules extending Clinical Gastroenterology and Hepatology 2016;-:-–-
ELECTRONIC IMAGE OF THE MONTH, continued to neighboring organs such as liver, omentum, and duodenum.1 It often is misdiagnosed as GB cancer because of the overlapping features between the two. In less than 3% of patients GB cancer can co-exist with XGC.1 Radiology can help to differentiate between the two but histopathology is the gold standard. The treatment is simple cholecystectomy but often ends up in partial cholecystectomy as a result of adhesions. The pathogenesis of XGC is not elucidated clearly. The hypothesized mechanisms are as follows: (1) extravasation of bile into the GB wall through mucosal ulcers or rupture of the Rokitansky–Aschoff sinuses,1 (2) infectious etiology considering histologic similarity with xanthogranulomatous pyelonephritis and malakoplakia,3 and (3) role of cell-mediated immunity such as a delayed hypersensitivity response.5
References 1.
e2
Deng YL, Cheng NS, Zhang SJ, et al. Xanthogranulomatous cholecystitis mimicking gallbladder carcinoma: an analysis of 42 cases. World J Gastroenterol 2015;21:12653–12659.
2.
Guzman-Valdivia G. Xanthogranulomatous cholecystitis: 15 years’ experience. World J Surg 2004;28:254–257.
3.
Mori M, Watanabe M, Sakuma M, et al. Infectious etiology of xanthogranulomatous cholecystitis: immunohistochemical identification of bacterial antigens in the xanthogranulomatous lesions. Pathol Int 1999;49:849–852.
4.
McCoy JJ Jr, Vila R, Petrossian G, et al. Xanthogranulomatous cholecystitis: report of two cases. J S C Med Assoc 1976; 72:78–79.
5.
Nakashiro H, Haraoka S, Fujiwara K, et al. Xanthogranulomatous cholecystis. Cell composition and a possible pathogenetic role of cell-mediated immunity. Pathol Res Pract 1995; 191:1078–1086.
Conflicts of interest The authors disclose no conflicts. © 2016 by the AGA Institute 1542-3565/$36.00 http://dx.doi.org/10.1016/j.cgh.2016.07.027
15-year-old boy with a history of autism spectrum disorder was admitted to our hospital with abdominal pain. He originally had presented 2 weeks earlier with abdominal pain but was discharged after ruling out appendicitis by an ultrasound. He returned with ongoing pain in the right upper quadrant (RUQ) with postprandial vomiting and fever. Vitals were as follows: blood pressure, 101/57 mm Hg; pulse rate, 104/ min; temperature, 99.1 F; and body mass index, 18.3 kg/ m2. Abdominal examination showed a firm and tender RUQ with negative Murphy’s sign. The patient’s white blood cell count was 15,800 cells/ mm3 with 78.3% neutrophils, hemoglobin level was 11.8 g/ dL, total bilirubin level was 2.9 mg/dL with direct bilirubin of 2.1 mg/dL, aspartate aminotransferase level was 29 U/L, alanine aminotransferase level was 32 U/L, alkaline phosphatase level was 317 U/L, g-glutamyltransferase level was 309 U/L, international normalized ratio was 1.43, and C-reactive protein level was 300 mg/L. All other laboratory results were within normal limits. RUQ ultrasound (Figure A) showed gallstones and a complex cystic area (10.3 6.8 7.8 cm) without internal vascularity, confirmed by a computed tomography scan of the abdomen (Figure A), which showed large multiloculated cystic lesion with septations in the region of the porta hepatis and gallbladder (GB) fossa measuring 8.2 7.5 10.0 cm with mass effect leading to Mirizzi syndrome.
A
The patient was managed with intravenous fluids and antibiotics. Endoscopic retrograde cholangiopancreatography showed a smooth common bile duct stricture with intrahepatic biliary dilation with dye filling the cystic mass (Figure B). Endoscopic ultrasound–guided fine-needle aspiration was negative for malignant cells and hence a frozen section was not performed. Aerobic and anaerobic cultures remained sterile. Subsequently, the patient underwent exploratory laparotomy with complete resection of the large multicystic mass (Figure C). Pathology showed a mass consistent with GB with severe acute and chronic inflammation and multiple granulomas (Figure C). He was discharged home after 16 days of hospitalization and was asymptomatic at his follow-up visit.
Discussion Xanthogranulomatous cholecystitis (XGC) is a rare variant of chronic cholecystitis characterized by destructive inflammation of the GB.1–3 XGC first was described by McCoy et al4 in 1976, representing approximately 10% of all cases of cholecystitis.2 With a slight male preponderance, XGC is seen rarely in young patients.2 The inflammation can be intense, with infiltration and fibrosis leading to asymmetric thickening of the GB wall and formation of multiple nodules extending Clinical Gastroenterology and Hepatology 2016;-:-–-
ELECTRONIC IMAGE OF THE MONTH, continued to neighboring organs such as liver, omentum, and duodenum.1 It often is misdiagnosed as GB cancer because of the overlapping features between the two. In less than 3% of patients GB cancer can co-exist with XGC.1 Radiology can help to differentiate between the two but histopathology is the gold standard. The treatment is simple cholecystectomy but often ends up in partial cholecystectomy as a result of adhesions. The pathogenesis of XGC is not elucidated clearly. The hypothesized mechanisms are as follows: (1) extravasation of bile into the GB wall through mucosal ulcers or rupture of the Rokitansky–Aschoff sinuses,1 (2) infectious etiology considering histologic similarity with xanthogranulomatous pyelonephritis and malakoplakia,3 and (3) role of cell-mediated immunity such as a delayed hypersensitivity response.5
References 1.
e2
Deng YL, Cheng NS, Zhang SJ, et al. Xanthogranulomatous cholecystitis mimicking gallbladder carcinoma: an analysis of 42 cases. World J Gastroenterol 2015;21:12653–12659.
2.
Guzman-Valdivia G. Xanthogranulomatous cholecystitis: 15 years’ experience. World J Surg 2004;28:254–257.
3.
Mori M, Watanabe M, Sakuma M, et al. Infectious etiology of xanthogranulomatous cholecystitis: immunohistochemical identification of bacterial antigens in the xanthogranulomatous lesions. Pathol Int 1999;49:849–852.
4.
McCoy JJ Jr, Vila R, Petrossian G, et al. Xanthogranulomatous cholecystitis: report of two cases. J S C Med Assoc 1976; 72:78–79.
5.
Nakashiro H, Haraoka S, Fujiwara K, et al. Xanthogranulomatous cholecystis. Cell composition and a possible pathogenetic role of cell-mediated immunity. Pathol Res Pract 1995; 191:1078–1086.
Conflicts of interest The authors disclose no conflicts. © 2016 by the AGA Institute 1542-3565/$36.00 http://dx.doi.org/10.1016/j.cgh.2016.07.027