AJR Teaching File: Intermittent Chronic Abdominal Pain, Fever, and Fatigue in a Middle-Aged Woman

Abstract

Commentary Intrahepatic biliary cystadenocarcinoma is a rare cystic hepatic neoplasm. It comprises about 0.41% of hepatic malignancies [1]. Intrahepatic biliary cystadenocarcinoma is solely intrahepatic in approximately 85% of the cases [2]. The tumor originates in the bile ducts and is lined by mucin-secreting columnar epithelial cells. It is a slow-growing tumor, occurring primarily in middle-aged women. It is believed that most intrahepatic biliary cystadenocarcinomas arise from cystadenomas, which are benign lesions with malignant potential [3, 4]. On pathologic examination, there is a band of closely bound spindle cells resembling ovarian stroma below the epithelium in this subgroup of intrahepatic biliary cystadenocarcinomas. Absence of this band is reported in another subgroup of intrahepatic biliary cystadenocarcinomas that are not associated with preexisting cystadenomas [5]. On imaging, both cystadenoma and cystadenocarcinoma are difficult to distinguish from other cystic lesions in the liver, especially when unilocular. Clinically, differentiation is also difficult because symptoms, when present, are vague and nonspecific. Detection and diagnosis of intrahepatic biliary cystadenocarcinoma rely heavily on radiologic findings. Recognizing the typical radiologic appearance is key so curative resection of a localized lesion can be performed [2]. On ultrasound, intrahepatic biliary cystadenocarcinoma presents as a complex cystic mass with thick walls that may have papillary projections, fluid-fluid levels, and internal septations [6–9]. Although sonography is better than CT for visualizing the internal patterns of the cysts, CT can better define the overall size, shape, and extent of the lesion [10]. AJR Teaching File: Intermittent Chronic Abdominal Pain, Fever, and Fatigue in a Middle-Aged Woman