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Gastric non-secreting neuroendocrine tumor and hypochlorhydria-related hypergastrinemia: a case report
© Biolato et al; licensee BioMed Central Ltd. 2013
Received: 21 July 2012
Accepted: 8 November 2012
Published: 22 February 2013
Zollinger–Ellison syndrome is characterized by recurrent peptic ulcers and diarrhea that result from gastrin-secreting neuroendocrine tumors of the gastrointestinal tract; nevertheless, severe hypergastrinemia may also have alternative pathogenetic explanations.
A 61-year-old woman of Caucasian origin presented with a history of epigastric pain and early satiety, severe hypergastrinemia (approximately 2000 pg/mL) and a neuroendocrine polyp in the corpus of her stomach. Chronic atrophic gastritis and intestinal metaplasia was present, but she denied use of acid suppressant drugs and the results of tests for Helicobacter pylori as well as gastric parietal cell and intrinsic factor antibodies were negative. She underwent a radical gastric tangential resection. Six months later, serum gastrin was still elevated despite lack of recurrence of tumor.
The clinical picture was suggestive for a hypochlorhydria-related hypergastrinemia with subsequent development of a non-secreting carcinoid. We suggest a periodic endoscopic follow-up in patients with severe hypochlorhydria-related hypergastrinemia in order to earlier detect neuroendocrine polyps.
Initially described in 1955 , Zollinger–Ellison syndrome is characterized by multiple and recurrent peptic ulcers and persistent diarrhea that result from gastrin-secreting neuroendocrine tumors (gastrinomas) of the gastrointestinal tract . Most gastrinomas are found in the pancreas, duodenum, or lymph nodes near the head of the pancreas, but they have also been found in other sites, and surgical resection is the antitumor treatment of choice . Although both hypergastrinemia and histological features of neuroendocrine tumor are criteria for the diagnosis of Zollinger–Ellison syndrome, these data may also have an alternative pathogenetic explanation.
A severe (>1000 pg/mL) elevation of fasting serum gastrin concentration is usually suggestive for Zollinger–Ellison syndrome; on top of this, the use of acid suppressant medication (both proton pump inhibitors or H2-receptor antagonists), the presence of H. pylori infection and autoimmune achlorhydric atrophic gastritis without or with pernicious anemia may lead to mild-to-moderate hypergastrinemia [4, 5]. In our case, the absence of symptoms of classical gastrinoma and the persistence of extremely elevated gastrin after radical tumor excision rule out the diagnosis of Zollinger–Ellison syndrome. The gastric biopsy performed six months after tumor resection showed chronic atrophic gastritis and intestinal metaplasia, with mild hyperplasia of antral G cells; this data, together with vitamin B12 deficiency anemia, suggest a hypochlorhydria-related hypergastrinemia. Although gastric parietal cell and intrinsic factor antibodies were both negative, cases of autoantibody-negative type A gastritis have been described . More than 100 cases of gastric carcinoids in patients with pernicious anemia are described in the literature. Because the hormone gastrin regulates several important cellular processes in the gastric epithelium including proliferation, apoptosis, migration, invasion, tissue remodeling and angiogenesis , we hypothesize that the unusual and extremely elevated levels of gastrin facilitated the occurrence of gastric neuroendocrine tumor in this patient . Some authors have suggested that small multiple gastric carcinoids associated with atrophic gastritis are indolent, despite patients having continuous hypergastrinemia [9, 10]; notwithstanding, a surgical approach in tumors larger than two cm seems to be cautious.
We suggest a periodic endoscopic follow-up in patients with severe (>1000 pg/mL) hypochlorhydria-related hypergastrinemia in order to earlier detect neuroendocrine polyps.
Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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