PMID- 35963905 OWN - NLM STAT- MEDLINE DCOM- 20221013 LR - 20221013 IS - 1534-4681 (Electronic) IS - 1068-9265 (Linking) VI - 29 IP - 12 DP - 2022 Nov TI - Long-Term Outcomes for Patients with Multiple Endocrine Neoplasia Type 1 and Duodenopancreatic Neuroendocrine Neoplasms. PG - 7808-7817 LID - 10.1245/s10434-022-12350-1 [doi] AB - BACKGROUND: Liver metastasis from duodenopancreatic neuroendocrine neoplasms (DP-NENs) is a major cause of mortality in multiple endocrine neoplasia type 1 (MEN1) patients, yet much of their natural history is unknown. METHODS: This longitudinal, retrospective cohort study analyzed all MEN1 patients with imageable functional (F) and nonfunctional (NF) DP-NENs (1990-2021) for liver metastasis-free survival (LMFS) and overall survival (OS). RESULTS: Of 138 patients, 85 (61.6%) had imageable DP-NENs (28 F, 57 NF), and the mean largest tumor size was 1.8 +/- 1.4 cm. Multifocality was present in 32 patients (37.7%). Surgery was performed for 49 patients (57.7%). During an 11-year median follow-up period (IQR, 6-17 years), 23 (27.1%) of the patients had liver metastasis, and 19 (22.4%) patients died. Death was attributed to liver metastasis in 60% of cases. The patients with F-DP-NENs versus NF-DP-NENs more often had liver metastasis (46.4% vs. 15.8%; p = 0.002) but had similar 10-year LMFS (80.9 vs. 87.0%; p = 0.44) and OS (82.7 vs. 94.3%; p = 0.69). The patients with NF-DP-NENs had surgery when their tumors were larger (p < 0.001). Tumor size was not associated with liver metastasis (p = 0.89). The average growth rate was 0.04 cm/year (SE, 0.02 cm/year; p = 0.01) during active surveillance for NF-DP-NENs (n = 38). Liver metastasis developed in four patients with tumors smaller than 2 cm. The risk of liver metastasis was independent of surgery (hazard ratio [HR], 0.78; 95% confidence interval [CI], 0.21-2.93; p = 0.72) and death (HR, 0.51; 95% CI, 0.08-3.06; p = 0.46). CONCLUSIONS: Although the observed outcomes in this study were better than historical data, small NF-DP-NENs still developed liver metastasis and liver metastasis remains a major cause of death. These results suggest that size as a sole criterion for surgery may be insufficient to predict tumor behavior. CI - (c) 2022. Society of Surgical Oncology. FAU - Liu, Jason B AU - Liu JB AD - Division of Endocrine Surgery, Department of Surgery, University of Pittsburgh, Pittsburgh, PA, USA. AD - Division of Gastrointestinal Surgical Oncology, Department of Surgery, University of Pittsburgh, Pittsburgh, PA, USA. FAU - Cai, Jinman AU - Cai J AD - Department of Surgery, University of Pittsburgh, Pittsburgh, PA, USA. FAU - Dhir, Mashaal AU - Dhir M AD - Division of Hepatobiliary Surgery and Surgical Oncology, Department of Surgery, Upstate University Hospital, Syracuse, NY, USA. FAU - Paniccia, Alessandro AU - Paniccia A AD - Division of Gastrointestinal Surgical Oncology, Department of Surgery, University of Pittsburgh, Pittsburgh, PA, USA. FAU - Zureikat, Amer H AU - Zureikat AH AD - Division of Gastrointestinal Surgical Oncology, Department of Surgery, University of Pittsburgh, Pittsburgh, PA, USA. FAU - Ramonell, Kimberly M AU - Ramonell KM AD - Division of Endocrine Surgery, Department of Surgery, University of Pittsburgh, Pittsburgh, PA, USA. FAU - McCoy, Kelly L AU - McCoy KL AD - Division of Endocrine Surgery, Department of Surgery, University of Pittsburgh, Pittsburgh, PA, USA. FAU - Carty, Sally E AU - Carty SE AD - Division of Endocrine Surgery, Department of Surgery, University of Pittsburgh, Pittsburgh, PA, USA. FAU - Yip, Linwah AU - Yip L AD - Division of Endocrine Surgery, Department of Surgery, University of Pittsburgh, Pittsburgh, PA, USA. yipl@upmc.edu. LA - eng PT - Journal Article DEP - 20220813 PL - United States TA - Ann Surg Oncol JT - Annals of surgical oncology JID - 9420840 SB - IM MH - Humans MH - *Liver Neoplasms/secondary MH - *Multiple Endocrine Neoplasia Type 1/complications/surgery MH - *Neuroendocrine Tumors/pathology MH - *Pancreatic Neoplasms/pathology MH - Retrospective Studies EDAT- 2022/08/14 06:00 MHDA- 2022/10/14 06:00 CRDT- 2022/08/13 23:19 PHST- 2022/06/09 00:00 [received] PHST- 2022/07/21 00:00 [accepted] PHST- 2022/08/14 06:00 [pubmed] PHST- 2022/10/14 06:00 [medline] PHST- 2022/08/13 23:19 [entrez] AID - 10.1245/s10434-022-12350-1 [pii] AID - 10.1245/s10434-022-12350-1 [doi] PST - ppublish SO - Ann Surg Oncol. 2022 Nov;29(12):7808-7817. doi: 10.1245/s10434-022-12350-1. Epub 2022 Aug 13.