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A Pancreatic Mass With Multiple Hepatic Lesions
ELECTRONIC IMAGE OF THE MONTH A Pancreatic Mass With Multiple Hepatic Lesions Howard H. W. Leung,* Carmen C. M. Cho,‡ and Anthony W. H. Chan§ *Faculty of Medicine, ‡Department of Imaging and Interventional Radiology, §Department of Anatomical and Cellular Pathology, Prince of Wales Hospital, The Chinese University of Hong Kong, Hong Kong
57-year-old man presented with jaundice, right upper-quadrant pain, and weight loss. Ultrasonography reviewed diffuse hypoechoic enlargement of the pancreas and hypoechoic lesions in the right lobe of the liver. Computed tomography showed focal heterogeneous enlargement of the pancreatic head, diffuse pancreatic swelling with hypodense capsule-like rim, and multiple hypoechoic-enhancing masses over bilateral liver lobes (Figure A). A clinical suspicion of pancreatic cancer with hepatic metastasis was raised. Endoscopic biopsy of pancreatic head mass found inflamed necrotic debris with ova of Clonorchis sinesis (Figure B). A targeted liver biopsy showed a fibroinflammatory process with plasma cell-rich chronic inflammatory infiltrate (Figure C), eosinophilic granulomas (Figure D), and increased IgG4-positive plasma cells (Figure E). Subsequent serology showed marked increases of serum IgG and IgG4 levels. A final diagnosis of hepatic inflammatory pseudotumors (IPTs) complicated by IgG4-related autoimmune pancreatitis and
A
Clonorchis infestation was established. A course of praziquantel was prescribed for Clonorchis infestation, and various immunosuppressants were given for IgG4related disease. However, the resolution of pancreatic and liver lesions was slow and fluctuated. Complete resolution finally was achieved after 3.5 years of immunosuppression (Figure F). C sinesis is the most common human liver fluke in East Asia and a well-known risk factor of cholangiocarcinoma.1 It is uncertain whether Clonorchis infestation contributes to tissue eosinophilia and eosinophilic granuloma in the liver biopsy of our patient because IgG4-related disease may have numerous tissue eosinophils.2,3 IgG4-related disease is an increasingly recognized fibroinflammatory disease characterized by tumefactive lesions, storiform fibrosis, IgG4-positive plasma cell-rich lymphoplasmacytic infiltrate, and an often increased serum IgG4 level. It affects virtually every organ: pancreas, salivary glands, liver, kidneys, lungs, lymph nodes, meninges, aorta, breast, prostate, Clinical Gastroenterology and Hepatology 2014;-:-–-
ELECTRONIC IMAGE OF THE MONTH, continued thyroid, pericardium, and skin.3 Hepatic involvement is accompanied very commonly by IgG4-related autoimmune pancreatitis, and has 2 major forms: IgG4-related cholangitis and hepatic IPT.2 IgG4–sclerosing cholangitis should be differentiated from primary sclerosing cholangitis because it is steroid-responsive and does not have an increased risk of cholangiocarcinoma. Hepatic IPT also should be distinguished from primary and metastatic liver masses to avoid unnecessary aggressive treatments. A substantial number of patients with IgG4related cholangitis/IPT underwent an unnecessary pancreatoduodenectomy or hepatic resection.2,4 Histologic examination is an essential investigation in the diagnosis of IgG4-related disease by identifying increased tissue IgG4-positive plasma cells. Although serum IgG4 level also is important, approximately 30% of patients with typical clinical and histopathologic features have normal serum IgG4 concentrations.3 A high index of suspicion of IgG4-related disease with proper clinical, pathologic, and serologic correlation is crucial to achieve the correct
e2
diagnosis of IgG4-related disease and provide appropriate treatment.
References 1.
Hong ST, Fang Y. Clonorchis sinensis and clonorchiasis, an update. Parasitol Int 2012;61:17–24.
2.
Bjornsson E, Chari ST, Smyrk TC, et al. Immunoglobulin G4 associated cholangitis: description of an emerging clinical entity based on review of the literature. Hepatology 2007;45: 1547–1554.
3.
Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med 2012;366:539–551.
4.
Ahn KS, Kang KJ, Kim YH, et al. Inflammatory pseudotumors mimicking intrahepatic cholangiocarcinoma of the liver; IgG4positivity and its clinical significance. J Hepatobiliary Pancreat Sci 2012;19:405–412.
Conflicts of interest The authors disclose no conflicts. © 2014 by the AGA Institute 1542-3565/$36.00 http://dx.doi.org/10.1016/j.cgh.2014.08.011
57-year-old man presented with jaundice, right upper-quadrant pain, and weight loss. Ultrasonography reviewed diffuse hypoechoic enlargement of the pancreas and hypoechoic lesions in the right lobe of the liver. Computed tomography showed focal heterogeneous enlargement of the pancreatic head, diffuse pancreatic swelling with hypodense capsule-like rim, and multiple hypoechoic-enhancing masses over bilateral liver lobes (Figure A). A clinical suspicion of pancreatic cancer with hepatic metastasis was raised. Endoscopic biopsy of pancreatic head mass found inflamed necrotic debris with ova of Clonorchis sinesis (Figure B). A targeted liver biopsy showed a fibroinflammatory process with plasma cell-rich chronic inflammatory infiltrate (Figure C), eosinophilic granulomas (Figure D), and increased IgG4-positive plasma cells (Figure E). Subsequent serology showed marked increases of serum IgG and IgG4 levels. A final diagnosis of hepatic inflammatory pseudotumors (IPTs) complicated by IgG4-related autoimmune pancreatitis and
A
Clonorchis infestation was established. A course of praziquantel was prescribed for Clonorchis infestation, and various immunosuppressants were given for IgG4related disease. However, the resolution of pancreatic and liver lesions was slow and fluctuated. Complete resolution finally was achieved after 3.5 years of immunosuppression (Figure F). C sinesis is the most common human liver fluke in East Asia and a well-known risk factor of cholangiocarcinoma.1 It is uncertain whether Clonorchis infestation contributes to tissue eosinophilia and eosinophilic granuloma in the liver biopsy of our patient because IgG4-related disease may have numerous tissue eosinophils.2,3 IgG4-related disease is an increasingly recognized fibroinflammatory disease characterized by tumefactive lesions, storiform fibrosis, IgG4-positive plasma cell-rich lymphoplasmacytic infiltrate, and an often increased serum IgG4 level. It affects virtually every organ: pancreas, salivary glands, liver, kidneys, lungs, lymph nodes, meninges, aorta, breast, prostate, Clinical Gastroenterology and Hepatology 2014;-:-–-
ELECTRONIC IMAGE OF THE MONTH, continued thyroid, pericardium, and skin.3 Hepatic involvement is accompanied very commonly by IgG4-related autoimmune pancreatitis, and has 2 major forms: IgG4-related cholangitis and hepatic IPT.2 IgG4–sclerosing cholangitis should be differentiated from primary sclerosing cholangitis because it is steroid-responsive and does not have an increased risk of cholangiocarcinoma. Hepatic IPT also should be distinguished from primary and metastatic liver masses to avoid unnecessary aggressive treatments. A substantial number of patients with IgG4related cholangitis/IPT underwent an unnecessary pancreatoduodenectomy or hepatic resection.2,4 Histologic examination is an essential investigation in the diagnosis of IgG4-related disease by identifying increased tissue IgG4-positive plasma cells. Although serum IgG4 level also is important, approximately 30% of patients with typical clinical and histopathologic features have normal serum IgG4 concentrations.3 A high index of suspicion of IgG4-related disease with proper clinical, pathologic, and serologic correlation is crucial to achieve the correct
e2
diagnosis of IgG4-related disease and provide appropriate treatment.
References 1.
Hong ST, Fang Y. Clonorchis sinensis and clonorchiasis, an update. Parasitol Int 2012;61:17–24.
2.
Bjornsson E, Chari ST, Smyrk TC, et al. Immunoglobulin G4 associated cholangitis: description of an emerging clinical entity based on review of the literature. Hepatology 2007;45: 1547–1554.
3.
Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med 2012;366:539–551.
4.
Ahn KS, Kang KJ, Kim YH, et al. Inflammatory pseudotumors mimicking intrahepatic cholangiocarcinoma of the liver; IgG4positivity and its clinical significance. J Hepatobiliary Pancreat Sci 2012;19:405–412.
Conflicts of interest The authors disclose no conflicts. © 2014 by the AGA Institute 1542-3565/$36.00 http://dx.doi.org/10.1016/j.cgh.2014.08.011