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Biliary tree gastrinomas in multiple endocrine neoplasia type 1 syndrome.
World J Gastroenterol. 2013 Dec 07; 19(45):8312-20.WJ

Abstract

AIM

To describe our patients affected with ectopic biliary tree gastrinoma and review the literature on this topic.

METHODS

Between January 1992 and June 2012, 28 patients affected by duodenopancreatic endocrine tumors in multiple endocrine neoplasia type 1 (MEN1) syndrome underwent surgery at our institution. This retrospective review article analyzes our experience regarding seventeen of these patients subjected to duodenopancreatic surgery for Zollinger-Ellison syndrome (ZES). Surgical treatment consisted of duodenopancreatectomy (DP) or total pancreatectomy (TP). Regional lymphadenectomy was always performed. Any hepatic tumoral lesions found were removed during surgery. In MEN1 patients, removal of duodenal lesions can sometimes lead to persistence or recurrence of hypergastrinemia. One possible explanation for this unfavorable outcome could be unrecognized ectopic localization of gastrin-secreting tumors. This study described three cases among the seventeen patients who were found to have an ectopic gastrinoma located in the biliary tree.

RESULTS

Seventeen MEN1 patients affected with ZES were analyzed. The mean age was 40 years. Fifteen patients underwent DP and two TP. On histopathological examination, duodeno pancreatic endocrine tumors were found in all 17 patients. Eighty-one gastrinomas were detected in the first three portions of the duodenum. Only one gastrinoma was found in the pancreas. The mean number of gastrinomas per patient was 5 (range 1-16). Malignancy was established in 12 patients (70.5%) after lymph node, liver and omental metastases were found. Three patients exhibited biliary tree gastrinomas as well as duodenal gastrinoma(s). In two cases, the ectopic gastrinoma was removed at the same time as pancreatic surgery, while in the third case, the biliary tree gastrinoma was resected one year after DP because of recurrence of ZES.

CONCLUSION

These findings suggest the importance of checking for the presence of ectopic gastrinomas in the biliary tree in MEN1 patients undergoing ZES surgery.

Authors+Show Affiliations

Francesco Tonelli, Francesco Giudici, Gabriella Nesi, Giacomo Batignani, Maria Luisa Brandi, Surgical Unit, Department of Surgery and Translational Medicine, University of Florence, 50014 Florence, Italy.Francesco Tonelli, Francesco Giudici, Gabriella Nesi, Giacomo Batignani, Maria Luisa Brandi, Surgical Unit, Department of Surgery and Translational Medicine, University of Florence, 50014 Florence, Italy.Francesco Tonelli, Francesco Giudici, Gabriella Nesi, Giacomo Batignani, Maria Luisa Brandi, Surgical Unit, Department of Surgery and Translational Medicine, University of Florence, 50014 Florence, Italy.Francesco Tonelli, Francesco Giudici, Gabriella Nesi, Giacomo Batignani, Maria Luisa Brandi, Surgical Unit, Department of Surgery and Translational Medicine, University of Florence, 50014 Florence, Italy.Francesco Tonelli, Francesco Giudici, Gabriella Nesi, Giacomo Batignani, Maria Luisa Brandi, Surgical Unit, Department of Surgery and Translational Medicine, University of Florence, 50014 Florence, Italy.

Pub Type(s)

Case Reports
Journal Article
Review

Language

eng

PubMed ID

24363522

Citation

Tonelli, Francesco, et al. "Biliary Tree Gastrinomas in Multiple Endocrine Neoplasia Type 1 Syndrome." World Journal of Gastroenterology, vol. 19, no. 45, 2013, pp. 8312-20.
Tonelli F, Giudici F, Nesi G, et al. Biliary tree gastrinomas in multiple endocrine neoplasia type 1 syndrome. World J Gastroenterol. 2013;19(45):8312-20.
Tonelli, F., Giudici, F., Nesi, G., Batignani, G., & Brandi, M. L. (2013). Biliary tree gastrinomas in multiple endocrine neoplasia type 1 syndrome. World Journal of Gastroenterology, 19(45), 8312-20. https://doi.org/10.3748/wjg.v19.i45.8312
Tonelli F, et al. Biliary Tree Gastrinomas in Multiple Endocrine Neoplasia Type 1 Syndrome. World J Gastroenterol. 2013 Dec 7;19(45):8312-20. PubMed PMID: 24363522.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Biliary tree gastrinomas in multiple endocrine neoplasia type 1 syndrome. AU - Tonelli,Francesco, AU - Giudici,Francesco, AU - Nesi,Gabriella, AU - Batignani,Giacomo, AU - Brandi,Maria Luisa, PY - 2013/04/29/received PY - 2013/06/29/revised PY - 2013/07/04/accepted PY - 2013/12/24/entrez PY - 2013/12/24/pubmed PY - 2014/6/13/medline KW - Biliary tree KW - Duodenopancreatectomy KW - Ectopic gastrinoma KW - Gastrinoma KW - Multiple endocrine neoplasia type 1 KW - Zollinger-Ellison Syndrome SP - 8312 EP - 20 JF - World journal of gastroenterology JO - World J. Gastroenterol. VL - 19 IS - 45 N2 - AIM: To describe our patients affected with ectopic biliary tree gastrinoma and review the literature on this topic. METHODS: Between January 1992 and June 2012, 28 patients affected by duodenopancreatic endocrine tumors in multiple endocrine neoplasia type 1 (MEN1) syndrome underwent surgery at our institution. This retrospective review article analyzes our experience regarding seventeen of these patients subjected to duodenopancreatic surgery for Zollinger-Ellison syndrome (ZES). Surgical treatment consisted of duodenopancreatectomy (DP) or total pancreatectomy (TP). Regional lymphadenectomy was always performed. Any hepatic tumoral lesions found were removed during surgery. In MEN1 patients, removal of duodenal lesions can sometimes lead to persistence or recurrence of hypergastrinemia. One possible explanation for this unfavorable outcome could be unrecognized ectopic localization of gastrin-secreting tumors. This study described three cases among the seventeen patients who were found to have an ectopic gastrinoma located in the biliary tree. RESULTS: Seventeen MEN1 patients affected with ZES were analyzed. The mean age was 40 years. Fifteen patients underwent DP and two TP. On histopathological examination, duodeno pancreatic endocrine tumors were found in all 17 patients. Eighty-one gastrinomas were detected in the first three portions of the duodenum. Only one gastrinoma was found in the pancreas. The mean number of gastrinomas per patient was 5 (range 1-16). Malignancy was established in 12 patients (70.5%) after lymph node, liver and omental metastases were found. Three patients exhibited biliary tree gastrinomas as well as duodenal gastrinoma(s). In two cases, the ectopic gastrinoma was removed at the same time as pancreatic surgery, while in the third case, the biliary tree gastrinoma was resected one year after DP because of recurrence of ZES. CONCLUSION: These findings suggest the importance of checking for the presence of ectopic gastrinomas in the biliary tree in MEN1 patients undergoing ZES surgery. SN - 2219-2840 UR - https://www.unboundmedicine.com/medline/citation/24363522/Biliary_tree_gastrinomas_in_multiple_endocrine_neoplasia_type_1_syndrome_ L2 - http://www.wjgnet.com/1007-9327/full/v19/i45/8312.htm DB - PRIME DP - Unbound Medicine ER -