<p>We report a 50-year-old man who was referred for evaluation of suspected hepatic sarcoidosis after non-caseating granulomas were incidentally identified on the liver surface during surgery. Laboratory tests showed a cholestatic pattern with elevated alkaline phosphatase and gamma-glutamyl transpeptidase, mild thrombocytopenia, and increased angiotensin-converting enzyme and soluble interleukin-2 receptor levels. Contrast-enhanced computed tomography demonstrated marked surface irregularity and reticular hypoattenuated linear structures within the liver. Gadoxetic acid–enhanced magnetic resonance imaging and contrast-enhanced ultrasonography revealed non-enhancing septum-like structures, whereas the intervening hepatic parenchyma showed preserved contrast uptake in the hepatobiliary phase of magnetic resonance imaging and in the post-vascular phase of contrast-enhanced ultrasonography. Upper gastrointestinal endoscopy identified esophagogastric varices. Liver biopsy showed preserved hepatic architecture without diffuse parenchymal inflammation. Azan staining demonstrated spatially heterogeneous portal fibrosis, with marked fibrous expansion in two portal tracts and mild fibrous expansion in several other portal tracts. Neither portal–portal nor portal–central bridging fibrosis was identified, and regenerative nodules surrounded by fibrous septa were not evident. Non-caseating epithelioid granulomas containing multinucleated giant cells were observed within fibrotic portal tracts. The patient was diagnosed with hepatic and splenic sarcoidosis complicated by portal hypertension without histologically established cirrhosis. This case highlights that portal tract–predominant, spatially heterogeneous fibrosis in hepatic sarcoidosis may contribute to portal hypertension and distinctive reticular hepatic imaging findings, even in the absence of histologically established cirrhosis.</p>

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Hepatic sarcoidosis presenting with portal hypertension without histologically established cirrhosis and reticular hepatic imaging features

  • Kenta Yamamoto,
  • Siena Itoh,
  • Kazuki Sahara,
  • Shinya Yokoyama,
  • Takanori Ito,
  • Norihiro Imai,
  • Yoji Ishizu,
  • Takeshi Yamamura,
  • Takashi Honda,
  • Hiroki Kawashima

摘要

We report a 50-year-old man who was referred for evaluation of suspected hepatic sarcoidosis after non-caseating granulomas were incidentally identified on the liver surface during surgery. Laboratory tests showed a cholestatic pattern with elevated alkaline phosphatase and gamma-glutamyl transpeptidase, mild thrombocytopenia, and increased angiotensin-converting enzyme and soluble interleukin-2 receptor levels. Contrast-enhanced computed tomography demonstrated marked surface irregularity and reticular hypoattenuated linear structures within the liver. Gadoxetic acid–enhanced magnetic resonance imaging and contrast-enhanced ultrasonography revealed non-enhancing septum-like structures, whereas the intervening hepatic parenchyma showed preserved contrast uptake in the hepatobiliary phase of magnetic resonance imaging and in the post-vascular phase of contrast-enhanced ultrasonography. Upper gastrointestinal endoscopy identified esophagogastric varices. Liver biopsy showed preserved hepatic architecture without diffuse parenchymal inflammation. Azan staining demonstrated spatially heterogeneous portal fibrosis, with marked fibrous expansion in two portal tracts and mild fibrous expansion in several other portal tracts. Neither portal–portal nor portal–central bridging fibrosis was identified, and regenerative nodules surrounded by fibrous septa were not evident. Non-caseating epithelioid granulomas containing multinucleated giant cells were observed within fibrotic portal tracts. The patient was diagnosed with hepatic and splenic sarcoidosis complicated by portal hypertension without histologically established cirrhosis. This case highlights that portal tract–predominant, spatially heterogeneous fibrosis in hepatic sarcoidosis may contribute to portal hypertension and distinctive reticular hepatic imaging findings, even in the absence of histologically established cirrhosis.