STS 2021

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[E-poster]

[E64] Mixed Adenoneuroendocrine Carcinoma Of The Ampulla Of Vater: Three Case Reports And A Literature Review
Min Kyu SUNG1 , Woohyung LEE*1 , Sarang HONG1 , Yejong PARK1 , Bong Jun KWAK1 , Ki Byung SONG1 , Jae Hoon LEE1 , Dae Wook HWANG1 , Song Cheol KIM1
1 Division Of Hepatobiliary And Pancreatic Surgery, Department Of Surgery, Asan Medical Center, University Of Ulsan College Of Medicine, REPUBLIC OF KOREA

Background : Introduction : Mixed adenoneuroendocrine carcinoma (MANEC) is defined as a tumor with a mixture of adenocarcinoma components and neuroendocrine neoplasm components. MANEC has two components, each accounting for at least 30% of all tumors. MANEC is sometimes located in the ampulla of Vater, a very rare location compared to other organs; thus, treatment and prognosis have not been established. We reported three cases of MANEC that occurred in the ampulla of Vater.

Methods : Case Presentation: The patients were a 65-year-old female, a 73-year-old female, and an 81-year-old male. Through the preoperative endoscopic biopsy, only the first patient was diagnosed with MANEC. The other two patients were diagnosed with adenocarcinoma in the ampulla of Vater. All three patients showed no distant metastasis at the time of diagnosis, so PPPD(pyrolus-preserving pancreaticoduodenectomy) was performed. Leucovorin plus 5-fluorouracil was selected as the adjuvant chemotherapy.

Results : Survival outcome: A 65-year-old woman (T3bN2, stage IIIB, the mitotic rate was 8/10 HPFs, Ki-67 labeling index 86% and CD56 staining positive) is still alive without recurrence for 1 year after adjuvant chemotherapy. A 73-year-old female (T2N1, stage IIIA, Ki-67 labeling index 90% and chromogranin positive and synaptophysin positive) who received adjuvant chemotherapy showed recurrences in the liver and para-aortic lymph nodes 4 months later. The total overall survival was 17 months from the date of operation. The 81-year-old male (T3aN1, stage IIIA, the mitotic rate was 36/10 HPFs, Ki-67 labeling index 80%) did not receive adjuvant chemotherapy due to his old age but has been still alive without recurrence after 5 years.

Conclusions : Conclusions: Each of the three patients had a different clinical course. In general, difficulty in preoperative diagnosis, risk of early recurrence, and poor disease course are the main hallmarks of MANEC arising from the ampulla of Vater. However, one of the patients (81-year-old male) in this case report survived despite not receiving adjuvant chemotherapy due to his old age. Therefore, it is important to establish a careful treatment strategy for MANEC arising from the ampulla of Vater.



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