Raptis D, Savvides E, Langas G, Chatzimavroudis G, Papaziogas B
2nd Department of Surgery, "G. Gennimatas" Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece.
Hippokratia. 2021 Jul-Sep;25(3):141-144.
Neuroendocrine neoplasm (NEN) of the cystic duct (CD) is an extremely rare entity, with misty clinical manifestation and incidental, in most cases, diagnosis. Due to its rarity, several dilemmas arise concerning the optimal treatment of this type of malignancy.
We report two cases of histologically confirmed NENs of the CD from our institution. Furthermore, we present a literature review focusing on the treatment type and likelihood of recurrence. The two patients underwent laparoscopic cholecystectomy (CCE) due to cholelithiasis and were both diagnosed with well-differentiated Grade 1 (G1) NEN. The first patient did not undergo further treatment as the surgical margins were clear. Regarding the second patient, complementary resection of the CD remnant was performed since the histopathological diagnosis indicated positive surgical margins. Active postoperative surveillance was suggested, and both patients remain disease-free to date. In the literature, we identified 22 previous cases of NENs of CD. Since there are still no standard guidelines, various surgical plans were adopted, varying from simple CCE to hepatic lobectomy and Roux en Y hepaticojejunostomy. Postoperative surveillance is reported for up to four years. Regardless of the implicated treatment plan, no patient was diagnosed with recurrent malignancy and the mortality rate was very low (1/22).
We propose that cholecystectomy with ligation of the CD proximal to its junction with the common hepatic duct is an adequate oncological treatment for G1 NENs of the CD. When preoperative or perioperative suspicion for malignancy is made, a frozen section of the CD should be sent for pathological examination to confirm radical resection (R0). Nevertheless, there is a need for further research that could validate our findings. HIPPOKRATIA 2021, 25 (3):141-144.
胆囊管神经内分泌肿瘤（NEN）是一种极其罕见的疾病，临床表现模糊，多数情况下为偶然诊断。由于其罕见性，对于这类恶性肿瘤的最佳治疗方案出现了一些难题。
我们报告了本院两例经组织学确诊的胆囊管NEN病例。此外，我们对治疗类型和复发可能性进行了文献综述。两名患者因胆结石接受了腹腔镜胆囊切除术（CCE），均被诊断为高分化1级（G1）NEN。第一名患者由于手术切缘清晰，未接受进一步治疗。对于第二名患者，由于组织病理学诊断显示手术切缘阳性，对胆囊管残余部分进行了补充切除。建议术后进行积极监测，两名患者至今均无疾病复发。在文献中，我们找到了之前22例胆囊管NEN病例。由于仍没有标准指南，采用了各种手术方案，从简单的CCE到肝叶切除术和Roux-en-Y肝空肠吻合术不等。术后监测报告长达四年。无论采用何种治疗方案，均无患者被诊断为恶性肿瘤复发，死亡率非常低（1/22）。
我们建议，在胆囊管与肝总管交界处近端结扎胆囊管的胆囊切除术是治疗胆囊管G1 NEN的一种充分的肿瘤学治疗方法。当术前或围手术期怀疑有恶性肿瘤时，应将胆囊管冰冻切片送检进行病理检查，以确认根治性切除（R0）。然而，需要进一步的研究来验证我们的发现。《希波克拉底》2021年，25（3）：141 - 144。
