Zhang Ning, Li Yatong, Zhao Mengyun, Chang Xiaoyan, Tian Feng, Qu Qiang, He Xiaodong
Department of General Surgery.
Department of Pathology, Peking Union Medical College Hospital, Beijing, China.
Medicine (Baltimore). 2018 Sep;97(39):e12549. doi: 10.1097/MD.0000000000012549.
Sarcomatous intrahepatic cholangiocarcinoma is a rare histological variant of cholangiocarcinoma (ICC). Previous medical literature has not mentioned the prevalence of this kind of disease, but a poorer prognosis than that of ordinary ICC was indicated. The diagnosis of the sarcomatous ICC is established on histopathological and immunohistochemical examinations. In this article, we present a new case of a patient with sarcomatous ICC who had no radiographic sign of intrahepatic tumor preoperatively.
A 63-year-old man was noted with cholecystolithiasis and right upper abdominal pain. Liver function was within normal limits, although the gamma-glutamyl transpeptidase level was elevated. Serum carbohydrate antigen 19-9 level was elevated. Radiography showed atrophy of the left lobe of the liver, high-intensity signals on T1 weighted images, and low/high-intensity signals on T2 weighted images in hepatic ducts.
The preoperative diagnoses were hepatolithiasis, choledocholithiasis, and cholecystolithiasis.
Exploratory laparotomy, adhesion release, cholecystectomy, choledocholithotomy, and T tube drainage were performed. During the surgery, an ill-defined tumor was detected on the atrophic left lateral lobe of the liver. Hepatic left lateral lobectomy was performed to remove the mass.
The final diagnosis of sarcomatous ICC was made by histopathology after surgery. No evidence of local recurrence or distant metastasis was noted on imaging during follow-up.
Although rare, sarcomatous ICC does exist in patients presented with cholecystolithiasis and liver atrophy. Surgeons should be aware of the existence of sarcomatous ICC due to the poor prognosis. We recommend that multidisciplinary approaches may be key to improve prognosis, including adjuvant chemotherapy or radiotherapy.
肉瘤样肝内胆管癌是胆管癌（ICC）一种罕见的组织学变体。既往医学文献未提及此类疾病的患病率，但提示其预后比普通ICC更差。肉瘤样ICC的诊断基于组织病理学和免疫组化检查。在本文中，我们报告了一例术前无肝内肿瘤影像学征象的肉瘤样ICC患者的新病例。
一名63岁男性被发现患有胆囊结石和右上腹疼痛。肝功能正常，尽管γ-谷氨酰转肽酶水平升高。血清糖类抗原19-9水平升高。影像学检查显示肝左叶萎缩，肝内胆管在T1加权图像上呈高信号，在T2加权图像上呈低/高信号。
术前诊断为肝内胆管结石、胆总管结石和胆囊结石。
进行了剖腹探查、粘连松解、胆囊切除术、胆总管切开取石术和T管引流术。手术过程中，在萎缩的肝左外叶发现一个边界不清的肿瘤。行肝左外叶切除术以切除肿块。
术后经组织病理学确诊为肉瘤样ICC。随访期间影像学检查未发现局部复发或远处转移迹象。
尽管罕见，但肉瘤样ICC确实存在于患有胆囊结石和肝萎缩的患者中。由于预后较差，外科医生应意识到肉瘤样ICC的存在。我们建议多学科方法可能是改善预后的关键，包括辅助化疗或放疗。
