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弥漫性肝内胆管癌的尸检诊断

Autopsy diagnosis of diffuse intrahepatic cholangiocarcinoma.

作者信息

Maruyama Akihiro, Nishikawa Takahiro, Nagura Asuka, Kurobe Takuya, Yashika Jun, Nimura Yuho, Hu Leiwing, Yamaguchi Tomohiro, Kojima Iori, Nonogaki Koji

机构信息

Department of Gastroenterology, Daido Hospital, 9 Hakusui-cho, Minami-ku, Nagoya-shi, Aichi, 457-8511, Japan.

Department of Pathology, Daido Hospital, 9 Hakusui-cho, Minami-ku, Nagoya-shi, Aichi, 457-8511, Japan.

出版信息

Clin J Gastroenterol. 2025 Feb;18(1):154-160. doi: 10.1007/s12328-024-02052-4. Epub 2024 Oct 21.

Abstract

Intrahepatic cholangiocarcinoma (ICC), a severe liver cancer, makes up to 20% of all hepatic malignancies and is difficult to diagnose early due to its often asymptomatic nature. This case report documents a rare presentation of ICC with multiple diffuse nodules not previously recorded in medical literature. A 65-year-old man with no significant medical history presented with back pain, anorexia, and significant weight loss. Elevated tumor markers and enlarged lymph nodes were observed, though imaging did not reveal a primary liver mass. Diagnostic efforts, including computed tomography and positron emission tomography scans and biopsies of lymph nodes and bone marrow, suggested adenocarcinoma of unknown primary origin. A definitive diagnosis was only made post-mortem, revealing multiple diffuse nodules in the liver identified as ICC, marking a rare presentation without a primary mass. This case highlights the diagnostic challenges posed by atypical ICC manifestations, where typical imaging does not indicate a primary mass, delaying diagnosis and treatment. The findings emphasize the importance of considering ICC in differential diagnoses in cases of unknown primary adenocarcinoma with liver involvement. The discovery of ICC with diffusely infiltrative nodules underscores the necessity for comprehensive diagnostic evaluations in patients presenting with nonspecific systemic symptoms and abnormal liver findings.

摘要

肝内胆管癌(ICC)是一种严重的肝癌,占所有肝脏恶性肿瘤的20%,由于其通常无症状的特性,早期难以诊断。本病例报告记录了一例罕见的ICC表现,即出现多个弥漫性结节,此前医学文献中未记载过。一名无重大病史的65岁男性出现背痛、厌食和明显体重减轻。观察到肿瘤标志物升高和淋巴结肿大,尽管影像学检查未发现肝脏原发性肿块。包括计算机断层扫描、正电子发射断层扫描以及淋巴结和骨髓活检在内的诊断工作提示为原发性不明的腺癌。仅在尸检后才做出明确诊断,发现肝脏中有多个弥漫性结节,确诊为ICC,这是一种罕见的无原发性肿块的表现。该病例突出了非典型ICC表现所带来的诊断挑战,即典型影像学检查未显示原发性肿块,从而延误了诊断和治疗。这些发现强调了在原发性不明的腺癌累及肝脏的病例中,在鉴别诊断时考虑ICC的重要性。发现具有弥漫性浸润性结节的ICC强调了对出现非特异性全身症状和肝脏异常表现的患者进行全面诊断评估的必要性。

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