Synchronous presentation of hepatocellular carcinoma and intrahepatic cholangiocarcinoma: Report of an exceptional case with review of the literature

Introduction and importance: The occurrence of distinct synchronous hepatocellular carcinoma (HCC) and intrahepatic cholangiocarcinoma (CC) is extremely rare. Less than 50 cases have been reported in the literature. The aim of our study was to describe the clinicopathological features of this association.

Case presentation: A 75-year-old female patient with chronic hepatitis C cirrhosis presented with three hepatic nodules affecting segments IV, VIII and V during follow-up of her disease. Only the V-segment nodule was radiologically suspicious of malignancy (classified as LI-RADS5). These nodules were resected after discussion of the case in a multidisciplinary meeting. Histological examination showed that the nodules in segments VIII and V corresponded respectively to an HCC with immunohistochemistry showing HepPar1 (+), CK7(-) and CK19(-), and to an intrahepatic CC with immunohistochemistry showing HepPar1 (-), CK7(+) and CK19(+). The excision was radical. The post-operative course was uncomplicated. After a 6-month follow-up, the patient did not develop any locoregional recurrence or metastases.

Clinical discussion: Synchronous association of HCC and CC is very uncommon, and diagnosis is based on pathological and immunohistochemical examination. Infection with the HCV represents a major risk factor for simultaneous association. Synchronous presentation in HCV-infected individuals has been associated with a poorer prognosis compared with cases where only a single type of liver cancer is present.

Conclusion: The prognosis of this association is generally poor, notably due to the aggressive behavior of CC. Surgical resection remains the first-line treatment option, when possible, but comprehensive management of these complex cases requires a multidisciplinary approach tailored to each patient's specific circumstances.