Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, 23-3 Matsuoka, Eiheiji-cho, Yoshida-gun, Fukui, 910-1193, Japan.
Division of Diagnostic Pathology/Surgical Pathology, University of Fukui Hospital, Fukui, Japan.
Clin J Gastroenterol. 2021 Oct;14(5):1496-1502. doi: 10.1007/s12328-021-01474-8. Epub 2021 Jul 3.
Primary adenosquamous carcinoma (ASC) of the liver is a rare subtype of cholangiocarcinoma that comprises both adenocarcinoma and squamous cell carcinoma components. We report a 48-year-old woman with advanced primary ASC and small cell carcinoma of the liver who had extrahepatic metastasis and received multiple chemotherapy regimens. After first presenting with upper abdominal pain, imaging revealed a 10.2 × 9.5 cm mass in the right lobe of the liver with lymph node and lung metastases. A liver tumor biopsy revealed adenocarcinoma and squamous cell carcinoma components, leading to a diagnosis of advanced primary ASC of the liver. The tumor shrank with gemcitabine/cisplatin therapy; however, neuron-specific enolase (NSE) and CYFRA levels were increased and the tumor grew. Next, hepatic arterial infusion chemotherapy using 5-fluorouracil and cisplatin decreased NSE and CYFRA levels and suppressed tumor growth. However, due to tumor growth, she died 14 months post-initial diagnosis. Post-autopsy pathology revealed a mixture of CD56- and synaptophysin-positive small cell carcinoma component in addition to ASC. We report a rare advanced primary ASC with small cell carcinoma of the liver diagnosed at autopsy.
原发性腺鳞癌(ASC)是胆管癌的一种罕见亚型,由腺癌和鳞状细胞癌成分组成。我们报告了一例 48 岁女性,患有晚期原发性 ASC 和肝小细胞癌,伴有肝外转移,并接受了多种化疗方案。首次出现上腹痛后,影像学检查显示右肝叶有一个 10.2×9.5cm 的肿块,伴有淋巴结和肺转移。肝脏肿瘤活检显示腺癌和鳞状细胞癌成分,导致诊断为晚期原发性 ASC。吉西他滨/顺铂治疗后肿瘤缩小;然而,神经元特异性烯醇化酶(NSE)和细胞角蛋白 19 片段(CYFRA)水平升高,肿瘤生长。随后,使用氟尿嘧啶和顺铂进行肝动脉灌注化疗,降低了 NSE 和 CYFRA 水平并抑制了肿瘤生长。然而,由于肿瘤生长,她在初始诊断后 14 个月死亡。尸检后病理显示,除了 ASC 之外,还存在 CD56-和突触素阳性的小细胞癌成分。我们报告了一例罕见的晚期原发性 ASC 合并肝小细胞癌,经尸检诊断。