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A 66-year-old woman was diagnosed as primary biliary cholangitis (PBC) and was previously hospitalized for ascites and jaundice. She came to our hospital for further examination of the liver by needle biopsy, which showed interface hepatitis that mainly comprised lymphocytes and inflammatory infiltrates in the bile duct in the portal area. On the other hand, numerous intracytoplasmic inclusions that were positive for fibrinogen immunostaining were seen in the lobular area. Finally, we histologically diagnosed as PBC with fibrinogen storage disease (FSD). FSD is rare disease that leads to liver damage caused by abnormal fibrinogen storage in the endoplasmic reticulum of hepatocytes, with only four cases reported in Japan until now.

The patient was a 62-year-old male who underwent a high anterior resection for rectal cancer with multiple liver metastases in June 2004. After the operation, 66 courses of weekly hepatic arterial infusion(HAI)therapy of 5-FU/Leucovorin( LV)were performed. Thereafter 14 courses of FOLFOX 4, 5 courses of FOLFIRI and 5 courses of FOLFOX 4 therapy were also sequentially performed. As a result of the CT examination, which revealed a new metastatic lesion in the liver and lung metastases, combination chemotherapy consisting of UFT and HAI of low-dose CPT-11 was administered in July 2007. After 1 cycle of this therapy, metastatic liver and lung tumors showed a reduction rate of 8.5% and 27.0%, respectively, without any adverse events. The elevated serum CEA (2,055 ng/mL)and CA19-9 (924 U/mL) levels decreased to 623 ng/mL and 332U /mL, respectively, after 1 cycle of the treatment. The combination of oral UFT and HAI of CPT-11 may therefore be a useful treatment for patients after FOLFOX and FOLFIRI therapy.

The application of radiofrequency ablation (RFA) for liver metastasis of colorectal cancer has not yet acquired an established status in clinical cancer therapy research. Removing as much tumor tissue as possible is desirable, but some cases do not allow optimal surgical ablation due to general condition of the patient and tumor status. We introduced endoscopic RFA for liver cancer in 2003, and have applied the procedure to 6 cases with H1 or H2 liver metastases of colorectal cancer to which surgical ablation could not be applied due to the poor general health of patients. Mean tumor diameter was 22.9 mm, and mean number of tumors per patient was 1.2. Tumor location was: S4, n = 2; S5, n = 1; S4, n = 1; S7, n = 2; and S8, n = 1. Mean frequency of session was 3.0. No complications occurred in any cases, and no reoperations were required. Although no recurrence of tumors in the vicinity of ablation was observed, 2 cases of each lung metastasis and intrahepatic recurrence were identified. Intrahepatic recurrence underwent hepatic arterial infusion (HAI) chemotherapy for simultaneous metastatic hepatic tumors (H2) prior to RFA, and relapses occurred in the metastatic focus where the efficacy of HAI was observed. At this point, 2 deaths were reported, 1 each from cancer and other diseases, and mean duration of survival after the procedure was 451.2 days. These results indicate that endoscopic RFA with good local control should be an available treatment for cases involving colorectal cancer with metastasis to the liver in which surgical ablation is difficult to apply.

Clinically common oncologic emergencies associated with pancreatobiliary cancer are gastrointestinal bleeding caused by duodenal invasion of pancreatic carcinoma, severe duodenal obstruction due to pancreatic carcinoma, and acute cholangitis accompanied by obstructive jaundice in patients with biliary tract carcinoma. When a patient with gallbladder cancer presents with acute cholecysitis, emergency surgery is sometimes performed on the basis of the latter diagnosis. Emergency procedures can also be required in the perioperative management of pancreatobiliary cancer, for example, in biliary peritonitis caused by detachment of a percutaneous transhepatic biliary drainage (PTBD) tube and in ruptured pseudoaneurysm due to postoperative pancreatic or biliary leakage. Nonsurgical procedures are usually initially selected for oncologic emergencies associated with pancreatobiliary cancer, because patients are likely to develop severe organ dysfunction and it is difficult to access directly and remove the pancreas or biliary tract during emergency surgery. When systemic conditions improve, it is necessary to evaluate the degree of disease progression and systemic conditions, and if feasible, the primary lesion should be surgically resected. When performing emergency cholecystectomy in patients with acute cholecystitis, thorough intraoperative investigation of resected specimens is important, considering the possibility of concomitant gallbladder carcinoma, since thorough examination cannot be performed in such emergency settings. Furthermore, when cholangitis accompanies pancreatobiliary cancer, emergency drainage should be considered as sepsis can develop rapidly.