Department of General Surgery, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310009, Zhejiang Province, China.
Department of Pathology, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310003, Zhejiang Province, China.
World J Gastroenterol. 2020 Feb 14;26(6):686-695. doi: 10.3748/wjg.v26.i6.686.
Neuroendocrine neoplasms are rarely located in the gallbladder (GB), and carcinoid syndrome is exceedingly rare in patients with GB neuroendocrine neoplasms.
We report a case of GB neuroendocrine carcinoma (GB-NEC) in a 65-year-old man, who presented with flushing for 2 mo. Pathological specimens of the flushed skin revealed that mucin was deposited between the collagen bundles in the dermis. Computed tomography and magnetic resonance imaging indicated neoplasm in the GB with liver invasion and enlarged lymph nodes in the portacaval space. High fluorodeoxyglucose uptake was detected in lymph nodes in the portacaval space, but distant metastasis was not seen by positron emission tomography. Ultrasound-guided needle biopsy of the GB neoplasm was suggestive of high-grade NEC. Because of the functional characteristics of poorly differentiated NEC, cholecystectomy, resection of hepatic segments IVb and V, pancreaticoduodenectomy, and regional lymphadenectomy were performed. A diagnosis of poorly differentiated NEC was made by pathological findings and immunohistochemical staining data. Ki-67 index was > 80%. The patient refused adjuvant therapy and passed away in the 7 month.
Distinctive manifestation combined with imaging helps make correct preoperative diagnosis. Radical surgery and adjuvant chemotherapy might improve prognosis.
神经内分泌肿瘤很少发生于胆囊（GB），而胆囊神经内分泌肿瘤患者发生类癌综合征则极为罕见。
我们报告了一例 65 岁男性的胆囊神经内分泌癌（GB-NEC）病例，其表现为潮红 2 个月。潮红皮肤的病理标本显示，真皮胶原束之间有黏蛋白沉积。计算机断层扫描和磁共振成像提示胆囊有肿瘤，侵犯肝脏，并在门腔静脉间隙有增大的淋巴结。正电子发射断层扫描未发现远处转移，但在门腔静脉间隙的淋巴结中检测到氟脱氧葡萄糖高摄取。超声引导下的胆囊肿瘤针吸活检提示高级别 NEC。由于低分化 NEC 的功能特征，行胆囊切除术、肝段 IVb 和 V 切除术、胰十二指肠切除术和区域淋巴结切除术。病理发现和免疫组织化学染色数据提示为低分化 NEC，Ki-67 指数>80%。患者拒绝辅助治疗，于 7 个月后去世。
独特的临床表现结合影像学检查有助于做出正确的术前诊断。根治性手术和辅助化疗可能改善预后。
