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

Nayssem Khessairi; Inès Mallek; Taher Laabidi; Ahlem Lahmar; Dhouha Bacha; Sana Ben Slama

doi:10.1016/j.ijscr.2023.109140

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

Int J Surg Case Rep. 2024 Jan:114:109140. doi: 10.1016/j.ijscr.2023.109140. Epub 2023 Dec 17.

Authors

Nayssem Khessairi¹, Inès Mallek², Taher Laabidi³, Ahlem Lahmar⁴, Dhouha Bacha⁴, Sana Ben Slama⁴

Affiliations

¹ Department of Pathology, M. Slim University Hospital Center, Tunisia. Electronic address: Nayssemkhessairi@gmail.com.
² Department of Pathology, M. Slim University Hospital Center, Tunisia.
³ Department of Surgery, M. Slim University Hospital Center, Tunisia.
⁴ Department of Pathology, M. Slim University Hospital Center, Tunisia; University of Tunis El Manar, Faculty of Medicine of Tunis, Tunisia.

Abstract

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.

Keywords: Chronic liver disease; Hepatocellular carcinoma; Intrahepatic cholangiocarcinoma; Synchronous primary liver cancer.

Publication types

Case Reports