Peripheral cholangiocarcinoma: presentation, diagnosis, pathology and management

To report the clinical presentation, diagnosis and results of aggressive surgical management in patients with intrahepatic cholangiocarcinoma.

From February 1988 to June 1998, 21 patients underwent laparotomy with a 90% resectability rate (19 resections). The 19 liver resections included right trisegmentectomy in six patients, right lobectomy in five, wedge resection in four, left lobectomy in two, left trisegmentectomy in one and a lateral segmentectomy in one. Resection of the biliary confluence with reconstruction by a Roux en Y hepaticojejunostomy was performed in three patients.

Mild abdominal pain, weight loss and gastrointestinal disturbances were the most frequent clinical signs. Jaundice was present in only four patients. Pre-operative radiological investigations (abdominal ultrasound, computed tomography, arteriography) correlated with pathological findings in only 60% of cases. Pre-operative histological findings (fine-needle cytology, liver biopsy), available for 19 patients, did not always provide an accurate diagnosis. The mortality and morbidity rates were 5 and 47%, respectively. The median survival of resected patients was 18 months. Overall patient and tumour-free survival rates were 83 and 31% at 1 year, 33 and 16.5% at 2 years and 16.5 and 16.5% at 3 years in the resected group. Lymph-node spread, vascular invasion, positive margins and bilobar distribution were associated with a high recurrence rate and poor prognosis.

Despite the advanced stage of these tumours at presentation, patient survival can be improved by aggressive surgical resection. As intrahepatic cholangiocarcinoma usually develops in a non-cirrhotic liver, major hepatic resections to obtain disease-free margins can be performed with low mortality.