Endocrine Abstracts

Introduction: Chronic hypergastrinemia is no longer an uncommon phenomenon. Zollinger-Ellison syndrome [ZES], chronic atrophic gastritis [CAG] type A, proton pump inhibitors [PPI] therapy, or Helicobacter pylori-induced CAG type B are causes of hypergastrinemia. Serum gastrin levels >1000 pg/ml (> 0 times the upper limit of normal) and PH gastric below 2 is the diagnostic of ZES, but two -thirds of ZES patients have serum gastrin levels below 1000 pg/ml. We present the case of a 63 years old female with a history of abdominal pain, chronic diarrhea, heartburn, vomiting, duodenal peptic ulcer. Additional evaluation revealed: chromogranin A level 6.5-fold higher (465.7 ng/ml), but under PPI therapy taken for the digestive symptomatology, thus nonspecific, hyperparathyroidism (vitamin D deficiency, normocalcemia), chronic autoimmune thyroiditis with an euthyroid phase. At referral, the patient was in mild distress due to the chronic diarrhea with a clinical evaluation showing normal blood pressure, IMC–26 kg/m² with pale skin.

The patient was referred to gastroenterology department, where the digestive symptomatology was responsive to higher dose of PPI (pantoprazole 120 mg/day). Endoscopic features excluded the atrophy expressed in CAG-A, while multiple proeminent sessile lesions (PFL) and superficial ulcerations were found. The biopsy of multiple ulcers (<1 cm) in the duodenum II showed the absence of Helicobacter Pylori and a histologic appearance (HA) similar to well differentiated neuroendocrine tumors. The immunohistochemistry showed the presence of chromogranin A and KI-67 positive in epithelial cells, inconclusive. A second endoscopy with biopsy of PFL was made, while the echo-endoscopy revealed another tumor around 1 cm diameter in the duodenum I with a HA suggestive for malignancy. Due to the insufficient material another biopsy was recommended. Fasting serum gastrin values were 6.5-fold higher under PPI therapy, thus nonspecific. After vitamin D supplementation, we can not exclude the primary hyperparathyroidism: normal vitamin D, normocalcemia, still a high value of PTH at 135.6 pg/ml with densitometric diagnosis of osteoporosis at the forearm without fragility fractures. The screening for MEN1 showed normal prolactin and IGF 1 levels. The control of acid output was achieved with a dose reduction of pantoprazole at 80 mg/day. Because of the lack of disponibility of 68 Gallium-DOTATATE, it was recommended to perform an Octreoscan.

Conclusion: This case raises awareness that often medical practice shows lack of correlation between clinical and paraclinical evaluation making a disease diagnostic like ZESa real challenge.

Keywords: ZES, hypergastrinemia, hyperparathyroidism, duodenal ulcers.