Intrahepatic multicystic biliary hamartoma: report of a case

Abstract

Multicystic biliary hamartoma is a very rare hamartomatous nodule in the liver, which has recently been described as a new category of hepatic nodular lesion. We herein report the case of a 55-year-old man histopathologically diagnosed with this entity following surgery. A solitary multilocular lesion in the liver was pointed out by ultrasonography during a systemic examination for a positive HBs antigen. This nodule could not be definitively diagnosed by radiologic modalities, including computed tomography, magnetic resonance imaging and arteriography. The patient underwent a partial resection of the posterior segment of the liver. The nodule was a localized lesion which measured 5 x 3 cm at the widest point and displayed a honeycomb appearance. Histologically, it consisted of ductal structures, periductal glands, fibrous connective tissues containing blood vessels, and bile-like materials and xanthogranulomatous inflammation within some ducts. Liver parenchyma was not present in the nodule and the bile ducts were not dilated in the background liver. The ductal epithelium expressed biliary type cytokeratins (CK7 and 19) in immunohistochemical studies. These histopathological features were consistent with multicystic biliary hamartoma, and we discuss this rare case in detail in this report.

Multicystic Biliary Hamartoma of the Liver

“…Immunohistochemical stainings for mucin core protein (Muc) 1, Muc2, Muc5Ac, Muc6, and biliary type keratin (K) 7 and, K19 were performed. Similar to previous findings,1,3 the duct-lining epithelial cells were diffusely positive for K7, K19 and showed focal Muc1 expression, whereas they were negative for Muc2, Muc5Ac, and Muc6 (Fig. 2D).…”

“…All these features, except for liver protrusion, were observed in the present case; five of the eight previously reported MCBHs also did not protrude from the liver 1-4. Previous reports additionally showed that MCBH occurs not only on the liver's surface but also in the intrahepatic portion 3. Therefore, we ultimately diagnosed this lesion as MCBH.…”

Caroli's disease: A report of 14 patients and review of the literature

“…Caroli's syndrome is associated with cirrhosis, portal hypertension and periportal fibrosis . The two types are regarded as different stages of the same disease . Recurrent cholangitis is the most common clinical manifestation, and biliary colic and jaundice occur as a consequence of intrahepatic calculi formation .…”

“…14 The two types are regarded as different stages of the same disease. 12,15,16 Recurrent cholangitis is the most common clinical manifestation, and biliary colic and jaundice occur as a consequence of intrahepatic calculi formation. 17,18 Most symptomatic patients experience a significant low quality of life.…”

A case of unclassified multicystic biliary tumor with biliary adenofibroma features

“…The gross appearance of the multicystic lesion of our case resembled that of an intrahepatic multicystic biliary hamartoma. A multicystic biliary hamartoma is composed of several components; ducts, periductal glands and connective tissue, 12,13 whereas the multicystic lesion in our case was composed of neoplastic cysts and fibrous stroma only.…”