Usui Genki, Hashimoto Hirotsugu, Kusakabe Masashi, Shirota Go, Sugiura Yoshiya, Fujita Yuji, Satou Shouichi, Harihara Yasushi, Horiuchi Hajime, Morikawa Teppei
NTT Medical Center Tokyo, Tokyo, Japan.
Japanese Foundation for Cancer Research, Tokyo, Japan.
Int J Surg Pathol. 2019 Dec;27(8):900-906. doi: 10.1177/1066896919855766. Epub 2019 Jun 17.
Carcinosarcomas with elements of cholangiocarcinoma and sarcoma are rare and have a poor prognosis. The spreading pattern and radiological findings of these lesions remain unclear. A 74-year-old man presented with a high γ-glutamyl transferase level. Magnetic resonance imaging revealed dilation of the right intrahepatic and common bile ducts, consistent with an intraductal papillary neoplasm of the bile duct (IPNB), and diffusion-weighted imaging (DWI) indicated an area of high signal intensity in the intrahepatic bile duct. Bile duct biopsy yielded a small amount of atypical spindle cells, and the patient underwent a right hepatectomy. Microscopically, the tumor contained cholangiocarcinoma and sarcomatous components, including osteosarcoma and leiomyosarcoma, leading to a diagnosis of intrahepatic carcinosarcoma. The tumor spread primarily through the intrahepatic bile duct. An accurate radiological diagnosis of carcinosarcoma was challenging, given the apparent similarities with IPNB. The findings from DWI and pathology of a bile duct biopsy may assist with preoperative diagnosis.
具有胆管癌和肉瘤成分的癌肉瘤罕见,预后较差。这些病变的扩散模式和影像学表现仍不明确。一名74岁男性出现γ-谷氨酰转移酶水平升高。磁共振成像显示右肝内胆管和胆总管扩张,符合胆管内乳头状肿瘤(IPNB),弥散加权成像(DWI)显示肝内胆管有高信号区。胆管活检发现少量非典型梭形细胞,患者接受了右肝切除术。显微镜下,肿瘤包含胆管癌和肉瘤成分,包括骨肉瘤和平滑肌肉瘤,诊断为肝内癌肉瘤。肿瘤主要通过肝内胆管扩散。鉴于与IPNB有明显相似之处,对癌肉瘤进行准确的影像学诊断具有挑战性。DWI结果和胆管活检病理可能有助于术前诊断。