Murabayashi Toji, Kanno Yoshihide, Odaira Takashi, Koshita Shinsuke, Ogawa Takahisa, Kusunose Hiroaki, Sakai Toshitaka, Yonamine Keisuke, Miyamoto Kazuaki, Kozakai Fumisato, Endo Kazuki, Noda Yutaka, Sawai Takashi, Ito Kei
Department of Gastroenterology, Sendai City Medical Center, 5-22-1, Tsurugaya, Miyaginoku, Sendai, 983-0824, Japan.
Department of Pathology, Sendai City Medical Center, 5-22-1, Tsurugaya, Miyaginoku, Sendai, 983-0824, Japan.
Clin J Gastroenterol. 2023 Apr;16(2):310-316. doi: 10.1007/s12328-023-01763-4. Epub 2023 Jan 23.
We report the first case of bile duct mixed neuroendocrine-non-neuroendocrine neoplasm (MiNEN) that had a mucinous carcinoma component. An 88-year-old man with biliary obstruction was diagnosed as having distal bile duct cancer using imaging examinations and endoscopic biopsy. The patient received the best supportive care without surgical resection for 13 months until death. An autopsy revealed a bulky mass involving the distal bile duct and multiple metastases in intra-abdominal lymph nodes, the liver, and the lungs. The primary cancer was microscopically diagnosed as a MiNEN, which consisted of mucinous adenocarcinoma and large cell-type neuroendocrine carcinoma (NEC) components. Metastatic lesions in the liver and lungs were composed of only NEC with rich extracellular mucin without adenocarcinoma cells. Using electron microscopy and immunohistochemistry, it was proved that all NEC cells in both primary and metastatic lesions had amphicrine features. On the basis of pathological findings, we thought that the MiNEN was initially derived from a mucinous adenocarcinoma that dedifferentiated to amphicrine NEC cells with mucin production.
我们报告了首例具有黏液腺癌成分的胆管混合性神经内分泌-非神经内分泌肿瘤（MiNEN）。一名88岁的男性因胆道梗阻接受影像学检查和内镜活检后被诊断为远端胆管癌。该患者未接受手术切除，接受最佳支持治疗13个月后死亡。尸检发现一个巨大肿块累及远端胆管，并在腹腔淋巴结、肝脏和肺出现多处转移。原发性癌症经显微镜诊断为MiNEN，由黏液腺癌和大细胞型神经内分泌癌（NEC）成分组成。肝脏和肺的转移病灶仅由富含细胞外黏液的NEC组成，无腺癌细胞。通过电子显微镜和免疫组织化学证实，原发性和转移性病灶中的所有NEC细胞均具有双分泌特征。基于病理结果，我们认为该MiNEN最初起源于黏液腺癌，其去分化为具有黏液分泌功能的双分泌NEC细胞。
