A case of multiple endocrine neoplasia: hyperparathyroidism, insulinoma, GRF-oma, hypercalcitoninaemia and intractable peptic ulceration.

Abstract

A 42-year-old woman with a family history of multiple endocrine neoplasia type 1 (MEN 1) presented with symptomatic hypoglycaemia and peptic ulceration. Investigation revealed an insulinoma, hyperparathyroidism, hypercalcitoninaemia with a positive pentagastrin stimulation test, acromegaly due to a GRF-oma, hyperprolactinaemia and normal serum gastrin levels. Five pancreatic tumours were removed at laparotomy and immunostaining was positive for insulin, calcitonin, somatostatin and glucagon. Post-operatively she developed elevated serum gastrin levels and gross peptic ulceration, despite H2-blockers, and died of gastro-intestinal haemorrhage suggesting that removal of the somatostatinoma may have allowed increased gastrin secretion from a gastrinoma. This case emphasizes the importance of measuring a wide variety of tumour marker peptides in MEN 1 and suggests that caution is required in interpretation of the pentagastrin stimulation test in such cases. Patients with MEN 1 and known peptic ulceration may require perioperative omeprazole treatment even if serum gastrin levels are normal.

• Authors: D E Price, S R Absalom, K Davidson, A Bolia, P R Bell, T A Howlett
• Journal: Clinical endocrinology (Clin Endocrinol (Oxf)) Vol. 37 Issue 2 Pg. 187-8 (Aug 1992) ISSN: 0300-0664 [Print] England
• PMID: 1356665 (Publication Type: Case Reports, Journal Article)

Chemical References

• Biomarkers, Tumor
• Calcitonin
• Growth Hormone-Releasing Hormone

Topics

• Adenoma (diagnosis)
• Adult
• Biomarkers, Tumor (analysis)
• Calcitonin (blood)
• Female
• Growth Hormone-Releasing Hormone (blood)
• Humans
• Hyperparathyroidism (diagnosis)
• Insulinoma (diagnosis)
• Multiple Endocrine Neoplasia (blood, complications, diagnosis)
• Pancreatic Neoplasms (diagnosis)
• Peptic Ulcer (etiology)
• Pituitary Neoplasms (diagnosis)