Hirano Shohei, Kubota Koji, Shimizu Akira, Notake Tsuyoshi, Ikehara Tomohiko, Kuroiwa Yudai, Nakamura Kenya, Soejima Yuji
Division of Gastroenterological, Hepato-Biliary-Pancreatic, Transplantation and Pediatric Surgery, Department of Surgery, Shinshu University School of Medicine.
Nihon Shokakibyo Gakkai Zasshi. 2025;122(1):59-67. doi: 10.11405/nisshoshi.122.59.
A 78-year-old male patient came to our hospital with a chief complaint of fever. Computed tomography revealed an indistinct tumor in the pancreatic head, along with dilatation of the bile duct and main pancreatic duct. An endoscopic transpapillary biopsy demonstrated adenocarcinoma in the glandular epithelium and a dense formation of quasi-round cells. Pathology results indicated positive CK AE1/AE3 and INSM-1, negative CD45, and a Ki67 index of about 80%, leading to a diagnosis of neuroendocrine carcinoma (NEC) Grade 3 of the pancreatic head. Consequently, a pancreatoduodenectomy was performed. Postoperative pathology revealed small cell NEC (SCNEC) at the pancreatic head, with infiltrative growth of atypical gland ducts around the bile ducts, indicating the presence of a well-differentiated adenocarcinoma. The adenocarcinoma contained in situ lesions and biliary intraepithelial neoplasia (BilIN), with SCNEC being contiguous. Therefore, the diagnosis was NEC originating from the extrahepatic bile duct, which invaded the pancreatic head. NEC of the extrahepatic bile duct is rare, accounting for approximately 0.2-2% of gastrointestinal neuroendocrine tumors, and it has a poor prognosis, similar to other gastrointestinal NECs, even when surgical treatment is performed. The patient remained an outpatient without recurrence 17 months postoperatively.
一名78岁男性患者因发热为主诉前来我院就诊。计算机断层扫描显示胰头有一界限不清的肿瘤，伴有胆管和主胰管扩张。内镜经乳头活检显示腺上皮为腺癌，并有密集的类圆形细胞形成。病理结果显示细胞角蛋白AE1/AE3和胰岛锌指蛋白-1（INSM-1）呈阳性，CD45呈阴性，Ki67指数约为80%，诊断为胰头神经内分泌癌（NEC）3级。因此，实施了胰十二指肠切除术。术后病理显示胰头为小细胞神经内分泌癌（SCNEC），胆管周围有非典型腺管浸润性生长，提示存在高分化腺癌。腺癌包含原位病变和胆管上皮内瘤变（BilIN），且与SCNEC相连。因此，诊断为起源于肝外胆管并侵犯胰头的神经内分泌癌。肝外胆管神经内分泌癌罕见，约占胃肠道神经内分泌肿瘤的0.2%-2%，预后较差，与其他胃肠道神经内分泌癌相似，即使进行手术治疗也是如此。该患者术后17个月门诊随访无复发。
