NEUROENDOCRINE NEOPLASM OF THE CYSTIC DUCT: REPORT OF TWO CASES AND REVIEW OF THE LITERATURE
Dimitrios Raptis*, Eliophotos Savvides, Georgios Langas, Nikolaos Beradze, Savvaidou Vasiliki, Chatzopoulos Dimitrios, Tzitiridou Maria, Grigoris Chatzimavroudis, Vasilios Papaziogas
2nd Surgical, Aristoteleio Panepistemio Thessalonikes, Thessalonike, Kentrikḗ Makedonía, Greece
Background: Neuroendocrine tumors (NETs) of the extrahepatic biliary tree are rare entities, with only, at about, one hundred cases having been reported in the literature. They arise from the embryonic neural crest cells and are potent malignant. Concerning the NETs of the cystic duct (CD), they are mostly incidental findings after cholecystectomy (CCE). Due to their rarity, several dilemmas and questions arise concerning the optimal surgical, systematic treatment and the efficient follow-up.
Case description: We report two cases of histologically confirmed NETs of the CD from our institution. Furthermore, we present the literature review in treatment and follow-up. Both of our cases were diagnosed incidentally as G1 NETs, after laparoscopic cholecystectomy due to cholelithiasis. The first patient was pathological evaluated as R0 resected and needed no further treatment except from follow-up. Concerning the second one, complementary resection of the CD remnant, with clear margins in the frozen section, was performed since the histopathological report initially indicated an R1 resection.
In the literature, we found 22 reported cases of NETs of the CD. Since there are still no common guidelines, variant surgical strategies are adopted to achieve clear surgical margins, varying from simple CCE to hepatic lobectomy and Roux-en-Y hepatojejunostomy. Post-operative surveillance was implied for up to 4 years. Regardless of the implicated treatment plan, no patient was diagnosed with recurrence. Furthermore, overall mortality rate was very low (1/22).
Conclusion: We propose that cholecystectomy with ligation of the CD proximal to its junction with the common hepatic duct (CHD) is oncological adequate for G1 NETs of the CD. When pre-, or peri-operative suspicion for malignancy is given, a frozen section of the specimen should be performed to confirm adequate resection. In cases of pathological incomplete resection, re-operation conventional or with minimally invasive approach is mandatory. In the current guidelines an instructive algorithm should be adopted.
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