Yamao Kentaro, Takenaka Mamoru, Imai Hajime, Nakai Atsushi, Omoto Shunske, Kamata Ken, Minaga Kosuke, Miyata Takeshi, Sakurai Toshiharu, Watanabe Tomohiro, Nishida Naoshi, Matsumoto Ippei, Takeyama Yosihumi, Chikugo Takaaki, Kudo Masatoshi
Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, Osaka-Sayama, Japan.
Oncology. 2017;93 Suppl 1:76-80. doi: 10.1159/000481236. Epub 2017 Dec 20.
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.
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.
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.
原发性硬化性胆管炎（PSC）是一种慢性胆汁淤积性肝病，其特征为胆管多发纤维性狭窄。PSC患者中超过40%的死亡与恶性肿瘤有关，包括胆管癌。原发性肝腺鳞癌（ASC）是胆管癌的一种罕见亚型，包含腺癌（AC）和鳞状细胞癌（SCC）成分，预后比其他胆管癌更差。我们报告了首例PSC患者发生肝ASC的病例。
一名28岁男性因对比增强计算机断层扫描（CE-CT）怀疑肝脓肿而前来诊断和治疗。他有溃疡性结肠炎和PSC病史。腹部CE-CT显示左叶有一个直径60毫米的环形肿块，中央坏死。磁共振成像显示在T1加权成像中肿块边界不清、信号强度低，在T2加权成像中为高信号强度肿块，内有散在的低信号强度区域。腹部超声显示肿瘤内有一个低回声成分，周围有弥漫性高回声区域。超声引导下活检及组织学检查显示肿瘤细胞具有鳞状和腺性分化。行左叶切除术。显微镜检查显示有两个成分，包括中度分化的AC和高分化的SCC。最终诊断为肝ASC。
这是首例报道的PSC患者发生肝ASC的病例。应认识到PSC患者不仅有发生一般胆管癌、肝细胞癌和转移性肝肿瘤的风险，还有发生ASC的风险。
