Long-term complete response of advanced hepatocellular carcinoma treated with multidisciplinary therapy including reduced dose of sorafenib: case report and review of the literature.

An 83-year-old man underwent computed tomography during a routine check-up due to a history of surgical treatment for pancreatic cancer. Two tumors were detected in the anterior segment of the liver. A needle biopsy of the larger tumor was performed, and pathological examination showed that the tumor was a poorly differentiated hepatocellular carcinoma. Resection was not performed considering the patient's poor physical condition. Thus, transcatheter arterial chemoembolization and radiofrequency ablation of the tumors were performed. Three months later, residual tumor of the larger lesion and multiple pulmonary metastases were detected. This time, continuous hepatic arterial infusion chemotherapy was performed. Although the pulmonary metastases markedly reduced, tumor thrombi appeared in the right portal vein on computed tomography. Finally, sorafenib was administered, which led to disappearance of the tumor thrombi and no other signs of recurrence 8 months after initiation of sorafenib on computed tomography. Although sorafenib administration has continued at reduced doses of 200 mg per day or less due to hypertension, complete response has persisted for the past 34 months. It is noteworthy that sorafenib has been given at reduced doses, but a long-term complete response is maintained in a patient who had portal tumor thrombi and distant metastasis. Herein, we present this rare case of advanced hepatocellular carcinoma controlled with reduced doses of sorafenib following multidisciplinary therapy, describe our single center experience with sorafenib use in patients with hepatocellular carcinoma, and review previous reports that focused on dose reduction of sorafenib.

Cystic tumor of the liver without ovarian-like stroma or bile duct communication: two case reports and a review of the literature.

We report two cases of cystic neoplasm of the liver with mucinous epithelium in which both ovarian-like stroma and bile duct communication were absent. The first case was a 41-year-old woman. She underwent right trisegmentectomy due to a multilocular cystic lesion, 15 cm in diameter, with papillary nodular components in the medial segment and right lobe. Histologically, arborizing papillae were seen in the papillary lesion. The constituent neoplastic cells had sufficient cytoarchitectural atypia to be classified as high-grade dysplasia. The second case was a 60-year-old woman. She underwent left lobectomy due to a unilocular cystic lesion, 17 cm in diameter, in the left lobe. Histologically, the cyst wall was lined by low columnar epithelia with slight cellular atypia. In both cases, neither ovarian-like stroma nor bile duct communications were found throughout the resected specimen. According to the most recent World Health Organization (WHO) classification in 2010, cystic tumors of the liver with mucinous epithelium are classified as mucinous cystic neoplasms when ovarian-like stromata are found, and as intraductal papillary neoplasm of bile duct when bile duct communication exists. Therefore, we diagnosed the cystic tumors as 'biliary cystadenoma' according to the past WHO classification scheme from 2000. We believe that the combined absence of both ovarian-like stroma and bile duct communication is possible in mucinous cystic tumors of the liver. Herein, we have described the clinicopathologic features of the two cases and reviewed past cases in the literature.

Concomitant pancreatic endocrine neoplasm and intraductal papillary mucinous neoplasm: a case report and literature review.

We report a case of concomitant pancreatic endocrine neoplasm (PEN) and intraductal papillary mucinous neoplasm (IPMN). A 74-year-old man had been followed-up for mixed-type IPMN for 10 years. Recent magnetic resonance images revealed an increase in size of the branch duct IPMN in the pancreas head, while the dilation of the main pancreatic duct showed minimal change. Although contrast-enhanced computed tomography and magnetic resonance imaging did not reveal any nodules in the branch duct IPMN, endoscopic ultrasound indicated a suspected nodule in the IPMN. A malignancy in the branch duct IPMN was suspected and we performed pylorus-preserving pancreatoduodenectomy with lymphadenectomy. The resected specimen contained a cystic lesion, 10 x 10 mm in diameter, in the head of the pancreas. Histological examination revealed that the dilated main pancreatic duct and the branch ducts were composed of intraductal papillary mucinous adenoma with mild atypia. No evidence of carcinoma was detected in the specimen. Incidentally, a 3-mm nodule consisting of small neuroendocrine cells was found in the main pancreatic duct. The cells demonstrated positive staining for chromogranin A, synaptophysin, and glucagon but negative staining for insulin and somatostatin. Therefore, the 3-mm nodule was diagnosed as a PEN. Since the mitotic count per 10 high-power fields was less than 2 and the Ki-67 index was less than 2%, the PEN was pathologically classified as low-grade (G1) according to the 2010 World Health Organization (WHO) criteria. Herein, we review the case and relevant studies in the literature and discuss issues related to the synchronous occurrence of the relatively rare tumors, PEN and IPMN.

Intrahepatic cholangiocarcinoma arising 33 years after excision of a choledochal cyst: report of a case.

We report a case of intrahepatic cholangiocarcinoma arising 33 years after excision of a choledochal cyst. A 61-year-old woman was admitted to our hospital complaining of fever. Thirty-three years ago she had undergone extrahepatic choledochal cystectomy and choledochojejunostomy for a choledochal cyst. Computed tomography showed a tumor in the anterior segment of the liver, extending to the posterior and medial segments and the right portal vein. Intrahepatic biliary stones were seen in the bile ducts. We performed extended right lobectomy. Microscopically, the tumor was cholangiocarcinoma. Most of the tumor area was composed of invasive adenocarcinoma but a carcinoma-in-situ component was also observed in some regions including the hilar bile duct, where an intrahepatic biliary stone was seen. This suggests that the cancer development could be related to intrahepatic cholestasis. Patients with choledochal cyst may have to be carefully followed up for more than 30 years even after diversion surgery.

Primary hepatic cancers with multiple pathologic features in a patient with hepatitis C: report of a case.

We report a case of multiple primary hepatic cancers exhibiting different pathologic features coexisting in a patient with chronic hepatitis C. Computed tomography showed 2 tumors in segment 8, 20 mm (S8-A) and 5 mm (S8-B) in diameter, and a 10-mm tumor in segment 6 (S6). Based on the images, the S8-A lesion was diagnosed as cholangiocellular carcinoma or combined hepatocellular carcinoma and cholangiocarcinoma (combined HCC-CC). The other 2 tumors were diagnosed as HCC. The patient underwent partial resections of segments 6 and 8. We found 2 more tumors (S8-C was 6 mm in diameter and S8-D was 4 mm) in the resected segment 8 specimen. Histopathologic examination revealed that the S8-A and S8-C tumors were combined HCC-CC, the S8-B and S6 lesions were scirrhous HCC, and the S8-D tumor was an early HCC. This is a very rare case in which different hepatic cancers with multiple pathologic features coexisted.