Cholangiolocarcinoma with Ductal Plate Malformation Pattern: A 6-Year Follow-Up

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INTRODUCTION: Cholangiolocarcinoma (CLC) with ductal plate malformation (DPM) is a rare primary liver cancer originating from the canals of Hering. It often exhibits intermediate behavior between hepatocellular carcinoma and intrahepatic cholangiocarcinoma. Diagnosing CLC with DPM is challenging due to overlapping imaging features with other liver malignancies.

CASE PRESENTATION: An 82-year-old man under surveillance for bladder cancer was incidentally found to have a liver nodule in segment 8. Over 6 years, the lesion grew from 10 mm to 41 mm and showed dynamic changes on imaging. Despite two inconclusive biopsies, a diagnosis of CLC with DPM was confirmed after a third biopsy and consultation with a specialized institution. The patient underwent a right hepatectomy, and pathological examination confirmed CLC with DPM. No evidence of recurrence was observed 19 months post-surgery.

CONCLUSIONS: This case underscores the importance of long-term follow-up and a multidisciplinary approach in managing rare hepatic malignancies. The clinical course provides valuable insights into the progression of CLC with DPM and may aid in diagnosing similar challenging cases.