Peripheral cholangiocarcinoma: presentation, diagnosis, pathology and management

Aims: To report the clinical presentation, diagnosis and results of aggress ive surgical management in patients with intrahepatic cholangiocarcinoma. Methods: From February 1988 to June 1998, 21 patients underwent laparotomy with a 90% resectability rate (19 resections). The 19 liver resections incl uded 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 r econstruction by a Roux en Y hepaticojejunostomy was performed in three pat ients. Results: Mild abdominal pain, weight loss and gastrointestinal disturbances were the most frequent clinical signs. Jaundice was present in only four p atients. Pre-operative radiological investigations (abdominal ultrasound, c omputed tomography, arteriography) correlated with pathological findings in only 60% of cases. Pre-operative histological findings (fine-needle cytolo gy, liver biopsy), available for 19 patients, did not always provide an acc urate diagnosis. The mortality and morbidity rates were 5 and 47%, respecti vely. The median survival of resected patients was 18 months. Overall patie nt and tumour-free survival rates were 83 and 31% at 1 year, 33 and 16.5% a t 2 years and 16.5 and 16.5% at 3 years in the resected group. Lymph-node s pread, vascular invasion, positive margins and bilobar distribution were as sociated with a high recurrence rate and poor prognosis. Conclusion: Despite the advanced stage of these tumours at presentation, pa tient survival can be improved by aggressive surgical resection. As intrahe patic cholangiocarcinoma usually develops in a non-cirrhotic liver, major h epatic resections to obtain disease-free margins can be performed with low mortality.