Category: Other - multiple endocrine neoplasia

Monitor: 21

21 - ZOLLINGER-ELLISON SYNDROME IN PATIENT WITH TYPE 1 MULTIPLE ENDOCRINE NEOPLASIA

Thursday, Apr 25
1:30 PM – 2:00 PM

Objective :

Multiple endocrine neoplasia type 1 (MEN1) includes a combination of more than 20 endocrine and non-endocrine tumors, with autosomal dominant Mendelian inheritance. The Zollinger-Elisson syndrome is a non-beta islet cell, gastrin-secreting tumor that stimulates gastrointestinal mucosal ulceration. 
We report the case of a 39-year-old female patient with history of epigastralgia, nausea, vomiting with blood streaks, attributed to acid peptic disease. Upon admission, she was constipated, with serum potassium of 2.7meq/L, calcium:13.8meq/L, phosphorus: 2.5meq/L; she was diagnosed with a metabolic ileus, requiring IV potassium. Due to abdominal pain, an abdominal ultrasound was performed, reporting dilation of the biliary ducts, the abdominal CT showed pancreatitis with multiple abdominal collections, nephrocalcinosis and bilateral pulmonary nodules.


Methods : na


Results : na


Discussion :

Gastroduodenoscopy reported esophagitis and an ulcerated mass in the Vatter ampulla, gastrin was 73.7ng/L. Hypercalcemia was found with secondary acute kidney failure that required hemodialysis due to contraindication of biphosphonates. Due to hypercalcemia, primary hyperparathyroidism screening was made, with PTH:1060pg/ml, vitamin D:24.18ng/ml. Thyroid ultrasound reported a nodular mass in the upper border right lobe.  The patient had history of 13 years of amenorrhea, with prolactin:1031ng/ml, FSH:0.411mIU/ml, LH:0.783mIU/ml, TSH:0.714uIU/ml, suggesting the presence of a prolactinoma. All these finding indicated the diagnosis of MEN-1. During hospitalization, the patient continued presenting upper gastrointestinal bleeding, requiring transfusion of red blood cells. The patient had cardiac arrest before being able to perform a sella turcica MRI. The autopsy revealed multiple intra-abdominal peripancreatic collections. Two tumors were found in the head and tail of the pancreas, a bronchial tumor, two parathyroid tumors and a pituitary tumor, all positive for neuro endocrine markers. The histological findings revealed a parathyroid carcinoma with hyperplastic nodules, invasive pituitary adenoma, and tumor located in pancreas and bronchus were neuroendocrine tumors degree 1 (NETG1). The gastric mucosa and the lower third of the esophagus were eroded and ulcerated with evidence of acute bleeding, attributing the Zollinger-Ellison syndrome as the main cause of death. 


Conclusion :

Parathyroid tumors are observed in 95% of patients with MEN1 with the manifestation of hypercalcemia caused by primary hyperparathyroidism. The Zollinger-Ellison syndrome is the most important cause of morbidity and mortality in patients with MEN1 associated with a massive digestive hemorrhage.

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Maria Alejandra Ramos Guifarro

Chief of Endocrinology
Hospital Escuela Universitario
Tegucigalpa, Francisco Morazan, Honduras

Chief of Endocrinology Hospital Escuela

Gerson Andino

Endocrinologist
Hospital Escuela Universitario

.

Daniel Andrés Guifarro Rivera

Medical Student
Universidad Nacional Autónoma de Honduras
Tegucigalpa, Francisco Morazan, Honduras

8th Year Medical Student

Mazlova Toledo

Pathologist
Hospital Escuela Universitario, Francisco Morazan, Honduras

.

Adoni Duarte

Pathology Resident
Hospital Escuela Universitario

.

Carlos Rivera