A case of giant cholangioblastic cholangiocarcinoma (12 kg) with left hepatectomy and hilar reconstruction: a surgical and pathological challenge.

Abstract

Introduction: Cholangioblastic cholangiocarcinoma is a rare variant of intrahepatic cholangiocarcinoma affecting young adults. Fewer than 30 cases have been reported. We describe a giant tumor in a 32-year-old man with morbid obesity (BMI of 54).

Case presentation: The patient presented with a 34 cm mass, pain, persistent vomiting, inability to tolerate oral intake, and a 30 kg weight loss. Biopsy confirmed cholangioblastic intrahepatic cholangiocarcinoma. As mass-effect symptoms and malnutrition precluded initial systemic treatment, he underwent high-risk palliative cytoreduction. A fresh 12 kg tumor was removed by left hepatectomy. Inadvertent hilar stapling in severely distorted anatomy required immediate portal vein and hepatic artery reconstruction and Roux-en-Y hepaticojejunostomy. Hepatic inflow was compromised for approximately 75 minutes. Estimated blood loss was 20 L. He was discharged on postoperative day 14.

Pathology: The specimen measured 53 × 33.5 × 22 cm and weighed 7.914 kg after fixation. Histology showed extensive venous and lymphovascular invasion. An involved transection margin and residual intrahepatic and nodal disease constituted an R2 resection; six of nine nodes were metastatic (pT2, pN1).

Discussion: This case illustrates the diagnostic, surgical, and anesthetic hazards of palliative resection for a giant hilar tumor, including the need for anatomical confirmation before hilar division and for preparedness for vascular reconstruction and massive hemorrhage.

Conclusion: Palliative cytoreduction relieved obstruction and enabled systemic therapy. The procedure was neither radical nor curative, and its potential symptomatic benefit must be balanced against substantial operative risk.