Primary Hepatic Adenosquamous Carcinoma Associated with Primary Sclerosing Cholangitis

Abstract

Introduction: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disorder characterized by multiple fibrotic strictures of the bile duct. More than 40% of deaths in PSC patients are related to malignant tumors, including cholangiocarcinoma. Primary hepatic adenosquamous carcinoma (ASC) is a rare subtype of cholangiocarcinoma containing adenocarcinoma (AC) and squamous cell carcinoma (SCC) components, with a poorer prognosis than other cholangiocarcinomas. We report the first case of a hepatic ASC in a patient with PSC. Case Report: A 28-year-old man was referred for diagnosis and treatment of a liver abscess suspected by contrast-enhanced computed tomography (CE-CT). He had a history of ulcerative colitis and PSC. Abdominal CE-CT revealed a 60-mm-diameter ring-shaped mass with central necrosis in the left lobe. Magnetic resonance imaging demonstrated a poorly circumscribed low-signal-intensity mass in T1-weighted imaging and a high-signal-intensity mass with a scattered low-signal-intensity area in T2-weighted imaging. Abdominal ultrasonography showed a hypoechoic component with a diffuse hyperechoic area in the tumor. Ultrasound-guided biopsy and histological examination showed tumor cells with both squamous and glandular differentiation. Left lobectomy was performed. Microscopic examination revealed 2 components, including moderately differentiated AC and well-differentiated SCC. The final diagnosis was hepatic ASC. Conclusion: This is the first reported case of hepatic ASC in a patient with PSC. Patients with PSC should be recognized as being at a risk of not only general cholangiocarcinoma, hepatocellular carcinoma, and metastatic liver tumor, but also ASC.

Unresectable primary hepatic adenosquamous carcinoma successfully treated with systemic and transcatheter hepatic arterial injection chemotherapies followed by conversion surgery: a case report and literature review

“…Owing to tissue necrosis associated with primary hepatic ASC, it can be easily confused with the liver abscess in the imaging data. Therefore, tissue pathology should be conducted to confirm the diagnosis of primary hepatic ASC [ 1 , 23 – 27 ]. In the present case, we suspected a liver abscess initially as the patient was febrile, had elevated inflammatory markers, and his CT scan showed a low-density area in the center of the tumor.…”

“…The available information on all the 72 cases (including our case) is summarized in Fig. 6 [ 1 , 2 , 6 , 7 , 16 – 21 , 23 – 46 ]. The patients in previously reported cases included 42 males and 30 females, with ages 28 through 85 years (median age: 65 years).…”

New Paradigm in Gastrointestinal Cancer Treatment

“…ASC contains both adenocarcinoma and squamous cell carcinoma components, tends to present more aggressive clinicopathologic features, and has a poorer prognosis than other cholangiocarcinomas. Yamao et al [14] first reported the first case of hepatic ASC in a patient with PSC. They concluded that patients with PSC should be recognized as being at risk of hepatic ASC, in addition to general cholangiocarcinoma, hepatocellular carcinoma (HCC), and metastatic liver tumor.…”