Ko Ardenne, MacKenzie Morgan, Chiu Kenrry, Yap Wan Wan, Melich George, MacKenzie Shawn
Department of Surgery, Royal Columbian Hospital, Fraser Health Authority, 330 East Columbia Street, New Westminster, British Columbia V3L 3W7, Canada.
Department of Pathology, Royal Columbian Hospital, Fraser Health Authority, 330 East Columbia Street, New Westminster, British Columbia V3L 3W7, Canada.
Int J Surg Case Rep. 2024 Dec;125:110604. doi: 10.1016/j.ijscr.2024.110604. Epub 2024 Nov 15.
Neuroendocrine neoplasm (NENs) make up approximately 2-3 % of gallbladder malignancies, while only 0.5 % of all NENs develop in the gallbladder. Most Gallbladder neuroendocrine neoplasms (GB-NENs) are discovered incidentally during pathological examinations post-cholecystectomy.
70-year-old male presents with an incidentally discovered 2.2 cm enhancing intraluminal soft tissue mass on abdominal CT scan. The mass demonstrates restricted diffusion on MR imaging, concerning for gallbladder malignancy. Radical cholecystectomy, confirms primary gallbladder neuroendocrine tumor (GB-NET). No adjuvant therapy was recommended at multidisciplinary cancer conference review. The patient is currently disease free at 18 months follow up.
The management of GB-NEN remains challenging, due to the lack of specific clinical manifestations and typical imaging features preoperatively. GB-NENs are usually asymptomatic, and the paucity of reported imaging characteristics makes prospective diagnosis of GB-NENs challenging. GB-NEN tend to be larger in size, demonstrating well defined, intact mucosa, with a thick rim of hyperintensity on diffusion weighted images (DWI). Distinguishing between gallbladder neuroendocrine carcinoma (GB-NEC) and gallbladder neuroendocrine tumor (GB-NET) on pathologic evaluation is essential in developing a treatment plan. GB-NETs have superior survival compared to GB-NECs. GB-NETs can be managed utilizing a cholecystectomy with portal lymphadenectomy +/- segment 4b/5 liver resection.
GB-NETs may achieve curative resection, if identified at an early disease stage.
神经内分泌肿瘤（NENs）约占胆囊恶性肿瘤的2%-3%，而所有NENs中只有0.5%发生于胆囊。大多数胆囊神经内分泌肿瘤（GB-NENs）是在胆囊切除术后的病理检查中偶然发现的。
一名70岁男性在腹部CT扫描时偶然发现一个2.2厘米的腔内强化软组织肿块。该肿块在磁共振成像上显示扩散受限，怀疑为胆囊恶性肿瘤。根治性胆囊切除术证实为原发性胆囊神经内分泌肿瘤（GB-NET）。在多学科癌症会议评估中未建议进行辅助治疗。患者在18个月的随访中目前无疾病。
由于术前缺乏特异性临床表现和典型影像学特征，GB-NEN的管理仍然具有挑战性。GB-NEN通常无症状，且报道的影像学特征较少，使得GB-NEN的前瞻性诊断具有挑战性。GB-NEN往往体积较大，表现为边界清晰、完整的黏膜，在扩散加权成像（DWI）上有厚的高信号边缘。在病理评估中区分胆囊神经内分泌癌（GB-NEC）和胆囊神经内分泌肿瘤（GB-NET）对于制定治疗计划至关重要。GB-NET的生存率优于GB-NEC。GB-NET可通过胆囊切除术加门静脉淋巴结清扫术+/-肝4b/5段切除术进行治疗。
如果在疾病早期发现，GB-NETs可能实现根治性切除。
