scholarly journals Zollinger Ellison Syndrome in a Patient with Multiple Endocrine Neoplasia Type 1: A Classic Presentation

2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Ishani Shah ◽  
Neil Vyas ◽  
Kambiz S. Kadkhodayan
Keyword(s):  
Multiple Endocrine Neoplasia ◽  
Multiple Endocrine Neoplasia Type ◽  
Chronic Diarrhea ◽  
Wide Spectrum ◽  
Pancreatic Neuroendocrine Tumor ◽  
Watery Diarrhea ◽  
Ulcer Disease ◽  
Endocrine Neoplasia ◽  
Zollinger Ellison Syndrome

Zollinger Ellison Syndrome (ZES) is characterized by a wide spectrum of conditions including severe gastroesophageal reflux disease, peptic ulcer disease, watery diarrhea, and weight loss. We present a case of a 60-year-old woman being evaluated for severe dyspepsia, vomiting, and chronic diarrhea, who was diagnosed to have ZES associated with a pancreatic neuroendocrine tumor, in the setting of multiple endocrine neoplasia (MEN) type 1. Although cases of ZES have been reported previously, we believe that our case is a classic presentation of ZES diagnosed on the basis of typical radiologic, endoscopic, and endosonographic features.

scholarly journals A Prospective Study of Gastric Carcinoids and Enterochromaffin-Like Cell Changes in Multiple Endocrine Neoplasia Type 1 and Zollinger-Ellison Syndrome: Identification of Risk Factors

2008 ◽  
Vol 93 (5) ◽  
pp. 1582-1591 ◽  
Author(s):  
Marc J. Berna ◽  
Bruno Annibale ◽  
Massimo Marignani ◽  
Tu Vinh Luong ◽  
Vito Corleto ◽  
...  
Keyword(s):  
Risk Factors ◽  
Multiple Endocrine Neoplasia ◽  
Multiple Endocrine Neoplasia Type ◽  
Clinical Laboratory ◽  
Gastric Carcinoids ◽  
Endocrine Neoplasia ◽  
Zollinger Ellison Syndrome ◽  
Ecl Cell ◽  
Cell Changes

Abstract Context: Multiple endocrine neoplasia type 1 (MEN1) patients frequently develop Zollinger-Ellison syndrome (ZES). These patients can develop proliferative changes of gastric enterochromaffin-like (ECL) cells and gastric carcinoids (ECL-cell tumors). ECL-cell changes have been extensively studied in sporadic ZES patients and can be precursor lesions of gastric carcinoids, but little is known about factors influencing their severity or development of carcinoids in MEN1/ZES patients. Objectives: Our objective was to prospectively analyze ECL-cell changes and gastric carcinoids (ECL-cell tumors) in a large series of MEN1/ZES patients to detect risk factors and deduct clinical guidelines. Setting and Patients: Fifty-seven consecutive MEN1/ZES patients participated in this prospective study at two tertiary-care research centers. Interventions and Outcome Measures: Assessment of MEN1, gastric hypersecretion, and gastroscopy with multiple biopsies was done according to a fixed protocol and tumor status. ECL-cell changes and α-human chorionic gonadotropin staining were assessed in each biopsy and correlated with clinical, laboratory, and MEN1 features. Results: ECL-cell proliferative changes were universally present, advanced changes in 53% and carcinoids in 23%. Gastric nodules are common and are frequently associated with carcinoids. Patients with high fasting serum gastrin levels, long disease duration, or a strong α-human chorionic gonadotropin staining in a biopsy are at higher risk for an advanced ECL-cell lesion and/or gastric carcinoid. Conclusions: Gastric carcinoids and/or advanced ECL-cell changes are frequent in MEN1/ZES patients, and therefore, regular surveillance gastroscopy with multiple routine biopsies and biopsies of all mucosal lesions are essential. Clinical/laboratory data and biopsy results can be used to identify a subgroup of MEN1/ZES patients with a significantly increased risk for developing gastric carcinoids, allowing development of better surveillance strategies.

scholarly journals The Antral Mucosa as a New Site for Endocrine Tumors in Multiple Endocrine Neoplasia Type 1 and Zollinger-Ellison Syndromes1

2001 ◽  
Vol 86 (5) ◽  
pp. 2236-2242 ◽  
Author(s):  
C. Bordi ◽  
V. D. Corleto ◽  
C. Azzoni ◽  
S. Pizzi ◽  
G. Ferraro ◽  
...  
Keyword(s):  
Multiple Endocrine Neoplasia ◽  
Endocrine Cells ◽  
Multiple Endocrine Neoplasia Type ◽  
Endocrine Tumors ◽  
Antral Mucosa ◽  
Men 1 ◽  
Endocrine Neoplasia ◽  
Zollinger Ellison Syndrome ◽  
Ecl Cell

Carcinoid tumors were identified in the antro-pyloric mucosa of four patients with multiple endocrine neoplasia type 1 (MEN-1)/Zollinger-Ellison syndrome, accounting for 8.7% of 46 patients with this condition examined by endoscopy and histology. In contrast, no tumors were found in the antral biopsies from 124 cases of sporadic Zollinger-Ellison syndrome (P < 0.001), indicating a prominent role for the MEN-1 gene defects in tumor development. Immunohistochemically the tumors did not express the hormones produced by antral endocrine cells (gastrin, somatostatin, serotonin). In contrast, two of them were diffusely immunoreactive for the isoform 2 of the vesicular monoamine transporter (VMAT-2), a marker specific for the gastric nonantral enterochromaffin-like (ECL) cells. In one of these patients a second antral VMAT-2-positive carcinoid was seen 21 months after the first diagnosis. The other two antral carcinoids were unreactive for VMAT-2. Multiple ECL cell tumors were found in the gastric body-fundus mucosa of the two patients with VMAT-2-positive, but not in those with VMAT-2-negative, antral carcinoids. In one case, the former tumors were diagnosed 22 months after the detection of the antral tumor. We conclude that the antral mucosa is an additional tissue that may harbor endocrine tumors in MEN-1 syndrome. These tumors did not express the phenotype of normal antral endocrine cells and, in at least two cases, were identified as ectopic ECL cell carcinoids.

scholarly journals Multiple Endocrine Neoplasia Type 1 with Concomitant Existence of Malignant Insulinoma: A Rare Finding

2021 ◽  
Vol 2021 ◽  
pp. 1-4
Author(s):  
Randhir Sagar Yadav ◽  
Ashik Pokharel ◽  
Deepshikha Gaire ◽  
Shumneva Shrestha ◽  
Ashbita Pokharel ◽  
...  
Keyword(s):  
Parathyroid Adenoma ◽  
Multiple Endocrine Neoplasia ◽  
Multiple Endocrine Neoplasia Type ◽  
Pancreatic Head ◽  
Pancreatic Neuroendocrine Tumor ◽  
Subtotal Parathyroidectomy ◽  
Malignant Insulinoma ◽  
Endocrine Neoplasia ◽  
Endocrine Neoplasia Type

Multiple endocrine neoplasia type 1 (MEN1) is a rare syndrome of autosomal dominant inheritance defined by co-occurrence of two or more tumors originating from the parathyroid gland, pancreatic islet cells, and/or anterior pituitary. Insulinoma which has an incidence of 0.4% is a rare pancreatic neuroendocrine tumor. Malignant insulinoma is extremely rare, while primary hyperparathyroidism is a common occurrence in MEN1. We present a case of MEN1 syndrome with 2.6 cm insulinoma in the pancreatic head and parathyroid adenoma in a 56-year-old female who presented with symptoms suggestive of hypoglycemia like multiple episodes of loss of consciousness for four years. Classical pancreaticoduodenectomy was carried out, and the postoperative period was uneventful. Later, subtotal parathyroidectomy was performed, which showed parathyroid adenoma. Patients presenting with features of hypoglycemia should be vigilantly assessed for the presence of a sinister pathology.

scholarly journals Síndrome de Zollinger-Ellison em pacientes com Neoplasia Endócrina Múltipla tipo 1 / Zollinger-Ellison Syndrome in pacients with multiple endocrine neoplasia type 1

2021 ◽  
Vol 4 (6) ◽  
pp. 28413-28420
Author(s):  
Júlia Carvalho Garcia De Assis ◽  
Pedro De Morais ◽  
Manuela Carvalho Garcia De Assis ◽  
Felipe Vaz De Paula ◽  
Nicole Rodrigues Martins ◽  
...  
Keyword(s):  
Multiple Endocrine Neoplasia ◽  
Multiple Endocrine Neoplasia Type ◽  
Endocrine Neoplasia ◽  
Zollinger Ellison Syndrome ◽  
Endocrine Neoplasia Type
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