PMID- 31818296 OWN - NLM STAT- MEDLINE DCOM- 20200501 LR - 20200501 IS - 1477-7819 (Electronic) IS - 1477-7819 (Linking) VI - 17 IP - 1 DP - 2019 Dec 9 TI - Case report: optimal tumor cytoreduction and octreotide with durable disease control in a patient with MEN-1 and Zollinger-Ellison syndrome-over a decade of follow-up. PG - 213 LID - 10.1186/s12957-019-1758-6 [doi] LID - 213 AB - BACKGROUND: Zollinger-Ellison syndrome (ZES) is a rare condition characterized by hypersecretion of gastrin by gastrinoma tumors leading to severe peptic ulcer disease with potential development of gastric carcinoid tumors. Herein, we report the clinical course of a 68-year-old patient with multiple endocrine neoplasia type 1 (MEN-1) who underwent several surgeries to ultimately undergo optimal tumor cytoreduction of locally advanced gastrinomas and symptomatic gastric carcinoids. The patient was subsequently maintained on octreotide long-acting release (LAR). This case report supports consideration for aggressive tumor cytoreduction and octreotide in similar patients with MEN-1-associated ZES for durable disease control and symptom management. CASE PRESENTATION: The patient is a 68-year-old male with multiple endocrine neoplasia type 1 (MEN-1), diagnosed in 1993 after presenting with recurrent renal calculi and hypercalcemia. Soon thereafter, he presented with symptoms and elevated gastrin levels suggestive of ZES prompting abdominal exploration with partial resection of the duodenum to remove gastrinoma tumor nodules. Within 4 years of the operation, he represented with intractable hypergastrinemia despite optimal medical management with peak gastrin levels exceeding 29,000 pg/mL, in 2006. In January 2007, the patient returned to the operating room for resection of regional peripancreatic and perigastric lymph nodes and enucleation of pancreatic body and tail gastrinoma tumors. Although his gastrin level decreased to 5000 pg/mL with resultant improvement of symptoms, in less than 2 years, he developed disease progression with obstructive symptomatology from enlarging gastric carcinoids and rising gastrin levels. In May of 2008, he underwent pancreaticoduodenectomy and near-total gastrectomy. Since June of 2008, the patient shows no demonstrable progression of disease and remains asymptomatic on LAR octreotide (30 mgs). Gastrin levels have been well controlled (range, 100-624 pg/mL; current 114 pg/mL). CONCLUSION: Success of this procedure in our case report highlights the potential role for optimal tumor cytoreduction and LAR octreotide to control disease progression in a patient with MEN-I and Zollinger-Ellison syndrome with locally advanced gastrinoma and secondary large gastric carcinoids. FAU - Daniels, Lynsey M AU - Daniels LM AD - Department of Surgery, Drexel University College of Medicine, 245 N. 15th Street, Suite 7150, Philadelphia, PA, 19102, USA. FAU - Khalili, Marian AU - Khalili M AD - Department of Surgery, Drexel University College of Medicine, 245 N. 15th Street, Suite 7150, Philadelphia, PA, 19102, USA. FAU - Morano, William F AU - Morano WF AD - Department of Surgery, Drexel University College of Medicine, 245 N. 15th Street, Suite 7150, Philadelphia, PA, 19102, USA. FAU - Simoncini, Michaela AU - Simoncini M AD - Department of Surgery, Drexel University College of Medicine, 245 N. 15th Street, Suite 7150, Philadelphia, PA, 19102, USA. FAU - Mapow, Beth C AU - Mapow BC AD - Department of Pathology, Drexel University College of Medicine, Philadelphia, PA, USA. FAU - Leaf, Andrea AU - Leaf A AD - Department of Medical Oncology, New York Harbor VA Medical Center, Brooklyn, NY, USA. FAU - Bowne, Wilbur B AU - Bowne WB AD - Department of Surgery, Drexel University College of Medicine, 245 N. 15th Street, Suite 7150, Philadelphia, PA, 19102, USA. Wbb002@gmail.com. LA - eng PT - Case Reports PT - Journal Article DEP - 20191209 PL - England TA - World J Surg Oncol JT - World journal of surgical oncology JID - 101170544 RN - 0 (Antineoplastic Agents, Hormonal) RN - RWM8CCW8GP (Octreotide) SB - IM MH - Aged MH - Antineoplastic Agents, Hormonal/*therapeutic use MH - Combined Modality Therapy MH - Cytoreduction Surgical Procedures/*methods MH - Humans MH - Male MH - Multiple Endocrine Neoplasia Type 1/complications/pathology/*therapy MH - Octreotide/*therapeutic use MH - Prognosis MH - Zollinger-Ellison Syndrome/complications/pathology/*therapy PMC - PMC6902565 OTO - NOTNLM OT - Carcinoid tumors OT - Cytoreduction OT - Gastrectomy OT - Gastrinoma OT - Multiple endocrine neoplasia type 1 OT - Octreotide OT - Pancreaticoduodenectomy OT - Zollinger-Ellison syndrome COIS- The authors declare that they have no competing interests. EDAT- 2019/12/11 06:00 MHDA- 2020/05/02 06:00 PMCR- 2019/12/09 CRDT- 2019/12/11 06:00 PHST- 2019/08/15 00:00 [received] PHST- 2019/11/25 00:00 [accepted] PHST- 2019/12/11 06:00 [entrez] PHST- 2019/12/11 06:00 [pubmed] PHST- 2020/05/02 06:00 [medline] PHST- 2019/12/09 00:00 [pmc-release] AID - 10.1186/s12957-019-1758-6 [pii] AID - 1758 [pii] AID - 10.1186/s12957-019-1758-6 [doi] PST - epublish SO - World J Surg Oncol. 2019 Dec 9;17(1):213. doi: 10.1186/s12957-019-1758-6.