PDF(Pubmed)
Abstract：
Intrahepatic cholangiocarcinoma (iCCA) with regional lymph node metastases, which lacks a well-delineated liver mass, may be misdiagnosed as a carcinoma of unknown primary (CUP) origin. The present study reports the case of a 69-year-old man initially diagnosed with CUP, who was incidentally found to have abdominal lymphadenopathy during ultrasonography (US). The clinical course from the time of lymphadenectomy and CUP diagnosis to iCCA detection after long-term follow-up is reported. A patient with a history of hypertensive renal disease presented with an incidental finding of enlarged abdominal lymph nodes in the perihepatic region on US. Abdominal contrast-enhanced computed tomography (CT) scan and magnetic resonance imaging (MRI) revealed two enlarged lymph nodes in the hepatoduodenal ligament. Exploratory laparotomy and lymphadenectomy were performed for diagnostic and therapeutic purposes, respectively. Poorly differentiated metastatic adenocarcinoma positive for cytokeratin 7 and negative for cytokeratin 20 was identified in two of the 22 lymph nodes. Postoperatively, a positron emission tomography/CT (PET/CT) scan was performed, which failed to locate the primary site. The diagnosis of CUP was confirmed based on clinical, radiological and histopathological characteristics. A sequential abdominal CT scan 48 months after lymphadenectomy revealed a faintly enhancing, intraductal polypoid mass with localized ductal dilatation in liver segment 3. MRI and PET/CT confirmed a mass in the left lobe of the liver. US-guided percutaneous needle biopsy confirmed the presence of moderately differentiated adenocarcinoma. The patient refused surgical treatment because of general weakness caused by Coronavirus disease 2019 infection. The patient received radical radiotherapy and underwent left hepatectomy after recovery of their performance status. Histopathological examination of the surgical specimen demonstrated prevailing fibrosis and mucin accumulation, with scattered cancer cells observed focally in the resected liver specimen owing to the effect of the radiotherapy. Consequently, a definitive diagnosis of primary adenocarcinoma of the intrahepatic bile duct was confirmed. The present report may improve understanding of the pathophysiology and clinical progression of iCCA, with a specific focus on the intraductal growth subtype.
摘要：
肝内胆管癌（iCCA）伴局部淋巴结转移，缺乏良好的肝脏质量，可能被误诊为不明原发癌（CUP）起源。本研究报告了一名69岁的男子最初被诊断患有CUP，在超声检查中偶然发现腹部淋巴结肿大（US）。报告了从淋巴结清扫术和CUP诊断到长期随访后iCCA检测的临床过程。有高血压肾病病史的患者在美国肝周区域偶然发现腹部淋巴结肿大。腹部对比增强计算机断层扫描（CT）扫描和磁共振成像（MRI）显示肝十二指肠韧带中有两个肿大的淋巴结。为了诊断和治疗目的，进行了剖腹探查术和淋巴结清扫术。分别。在22个淋巴结中的两个淋巴结中发现细胞角蛋白7阳性而细胞角蛋白20阴性的低分化转移性腺癌。术后,进行了正电子发射断层扫描/CT（PET/CT）扫描，无法找到主站点。根据临床证实了CUP的诊断，放射学和组织病理学特征。淋巴结清扫术后48个月的连续腹部CT扫描显示微弱增强，导管内息肉样肿块，肝3段局部导管扩张。MRI和PET/CT证实肝左叶有肿块。US引导经皮穿刺活检证实存在中分化腺癌。由于2019年冠状病毒感染引起的全身无力，患者拒绝手术治疗。患者接受了根治性放疗，并在恢复其表现状态后接受了左肝切除术。手术标本的组织病理学检查显示普遍存在的纤维化和粘蛋白积累，由于放疗的影响，在切除的肝脏标本中局部观察到分散的癌细胞。因此,确诊为原发性肝内胆管腺癌.本报告可以提高对iCCA的病理生理学和临床进展的理解。特别关注导管内生长亚型。
