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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.
World J Surg Oncol. 2019 Dec 09; 17(1):213.WJ

Abstract

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.

Authors+Show Affiliations

Department of Surgery, Drexel University College of Medicine, 245 N. 15th Street, Suite 7150, Philadelphia, PA, 19102, USA.Department of Surgery, Drexel University College of Medicine, 245 N. 15th Street, Suite 7150, Philadelphia, PA, 19102, USA.Department of Surgery, Drexel University College of Medicine, 245 N. 15th Street, Suite 7150, Philadelphia, PA, 19102, USA.Department of Surgery, Drexel University College of Medicine, 245 N. 15th Street, Suite 7150, Philadelphia, PA, 19102, USA.Department of Pathology, Drexel University College of Medicine, Philadelphia, PA, USA.Department of Medical Oncology, New York Harbor VA Medical Center, Brooklyn, NY, USA.Department of Surgery, Drexel University College of Medicine, 245 N. 15th Street, Suite 7150, Philadelphia, PA, 19102, USA. Wbb002@gmail.com.

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

31818296

Citation

Daniels, Lynsey M., et al. "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." World Journal of Surgical Oncology, vol. 17, no. 1, 2019, p. 213.
Daniels LM, Khalili M, Morano WF, et al. 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. World J Surg Oncol. 2019;17(1):213.
Daniels, L. M., Khalili, M., Morano, W. F., Simoncini, M., Mapow, B. C., Leaf, A., & Bowne, W. B. (2019). 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. World Journal of Surgical Oncology, 17(1), 213. https://doi.org/10.1186/s12957-019-1758-6
Daniels LM, et al. 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. World J Surg Oncol. 2019 Dec 9;17(1):213. PubMed PMID: 31818296.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - 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. AU - Daniels,Lynsey M, AU - Khalili,Marian, AU - Morano,William F, AU - Simoncini,Michaela, AU - Mapow,Beth C, AU - Leaf,Andrea, AU - Bowne,Wilbur B, Y1 - 2019/12/09/ PY - 2019/08/15/received PY - 2019/11/25/accepted PY - 2019/12/11/entrez PY - 2019/12/11/pubmed PY - 2020/5/2/medline KW - Carcinoid tumors KW - Cytoreduction KW - Gastrectomy KW - Gastrinoma KW - Multiple endocrine neoplasia type 1 KW - Octreotide KW - Pancreaticoduodenectomy KW - Zollinger-Ellison syndrome SP - 213 EP - 213 JF - World journal of surgical oncology JO - World J Surg Oncol VL - 17 IS - 1 N2 - 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. SN - 1477-7819 UR - https://www.unboundmedicine.com/medline/citation/31818296/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 L2 - https://wjso.biomedcentral.com/articles/10.1186/s12957-019-1758-6 DB - PRIME DP - Unbound Medicine ER -