Resected primary mucinous cholangiocarcinoma of the liver

Abstract

BackgroundMucinous cholangiocarcinoma (MC) is a very rare variant of intrahepatic cholangiocarcinoma. MC is characterized by rapid growth, widespread metastasis, and poor prognosis. We report a case of resected MC of the liver.Case presentationWe found a 13.6-cm hypovascular tumor in the left hepatic lobe of a 68-year-old man, which we initially diagnosed as a mass-forming intrahepatic cholangiocarcinoma. Left lobe and caudate resection was performed without major intraoperative or postoperative complications. He was discharged home on postoperative day 9 and had no recurrence for 6 months. Pathological examination showed a mucous lobulated tumor with abundant mucus in the cytoplasm and extracellular regions. After differential diagnosis that considered invasive intraductal papillary neoplasm of the bile duct and metastatic liver tumors from the digestive tract, this tumor was diagnosed as a cholangiocarcinoma rare variant: primary mucinous carcinoma of the liver.ConclusionAnalysis of previous reports suggests that primary MC of the liver could be classified into two subtypes: pure MC and combined hepatocellular carcinoma and MC. Notably, the latter has been reported only in patients with chronic liver disease, whereas the former has only been reported in patients with no underlying disease.

Primary small bowel adenocarcinoma with loss of nuclear expression of PMS2 after resection of mucinous cholangiocarcinoma

“…Mucinous cholangiocarcinoma is a rare type of intrahepatic cholangiocarcinoma, characterized by rapid growth, extensive metastasis and poor prognosis [ 1 , 3 , 4 ]. Proposed risk factors include a history of biliary disease, chronic hepatitis C and intrahepatic C. sinensis flukes [ 5 , 6 ].…”

“…Mucinous cholangiocarcinoma is an extremely rare form of intrahepatic cholangiocarcinoma that has been characterized by rapid growth, widespread metastasis and poor prognosis [ 1 ]. These tumors have been shown to be a part of the Lynch syndrome tumor spectrum, however, the role of DNA mismatch repair (MMR) deficiency in their development is still under research [ 2 ].…”

Pathological aspects of mucinous cholangiocarcinoma: A single‐center experience and systematic review

“…However, the radiologic findings cannot distinguish mucinous iCCAs from their conventional counterparts. 9,19 Moreover, it was not easy to distinguish between the IPNB and hepatocellular carcinoma with mucinous iCCA is some clinical settings. 7 By definition, mucinous carcinoma is a malignant neoplasm, in which extracellular mucinous components account for at least 50% of total tumor volume.…”

“…A total of 19 reported cases of mucinous cholangiocarcinoma published in the English literature [7][8][9][10][11][12][13][14][15][16][17][18][19] were retrieved (see Table S1). The clinicopathological features of © 2020 Japanese Society of Pathology and John Wiley & Sons Australia, Ltd these cases are summarized in Table 2.…”

“…The previously published literature revealed that this subtype is notorious for rapid growth, widespread metastasis, and poor prognosis than the conventional iCCA. [7][8][9] Due to its rarity, cautious histopathological examination is needed in order not to misdiagnose such unusual malignant neoplasm as secondary malignancy or other more common tumors. We performed a clinicopathologic study focusing on resected mucinous iCCA at our institution, which is a tertiary medical center in Bangkok, Thailand.…”