Gastric neuroendocrine neoplasms are rare. They are classified clinically in types 1 to 3. Gastric neuroendocrine neoplasms type 1 are observed in chronic atrophic gastritis as single or multiple small tumors. Their prognosis is excellent. Gastric neuroendocrine neoplasms type 2 are part of MEN 1 syndrome.
The aim is to describe the case of a patient with a gastric neuroendocrine neoplasm which was observed in a background of atrophic gastritis and an ectopic pheochromocytoma arising from the sympathetic chain with atypical behaviour not accompanied by arterial hypertension.
A patient, male aged 58 years, with megaloblastic anemia and atrophic gastritis was subjected to gastroscopy which showed a polypoid tumor >3 cm in diameter in the body of the stomach. A subtotal gastrectomy was performed and histology showed neuroendocrine dysplasia of the stomach. During surgery a subhepatic tumor was observed. In endocrinological investigation high urine VMA levels were observed, VMA being 10.9 mg/g creatinine, urine catecholamines were 392 microg/24 h and urine metanephrines 1300 microg/24 h as well as high blood gastrin levels 3130 pg/ml. Further investigation with magnetic resonance imaging showed a cystic lobulated mass measuring 4.3×5×4.5 cm with calcifications under the right side of the diaphragm. The mass was visualized in scintigraphy with 111In octreotide, a finding compatible with the presence of somatostatin receptors. The patient did not have arterial hypertension. The mass was excised and histology showed a paraganglioma deriving from the sympathetic chain. Two days after surgery the patient experienced an episode of hypotension and is now free of symptoms and well.
Conclusion: The extremely rare case of a paraganglioma not causing arterial hypertension in a patient with a neuroendocrine neoplasm of the stomach in a background of atrophic gastritis is described.
Prague, Czech Republic
24 - 28 Apr 2010
European Society of Endocrinology