A case of multiple endocrine neoplasia: hyperparathyroidism, insulinoma, GRF-oma, hypercalcitoninaemia and intractable peptic ulceration.
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.
Leicester Royal Infirmary, UK.
D E
DE
Price
S R
SR
Absalom
K
K
Davidson
A
A
Bolia
P R
PR
Bell
T A
TA
Howlett
D000236:Adenoma / Q000175:diagnosis; D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis; D002116:Calcitonin / Q000097:blood; D005260:Female; D013007:Growth Hormone-Releasing Hormone / Q000097:blood; D006801:Humans; D006961:Hyperparathyroidism / Q000175:diagnosis; D007340:Insulinoma / Q000175:diagnosis; D009377:Multiple Endocrine Neoplasia / Q000097:blood / Q000150:complications / Q000175:diagnosis*; D010190:Pancreatic Neoplasms / Q000175:diagnosis; D010437:Peptic Ulcer / Q000209:etiology; D010911:Pituitary Neoplasms / Q000175:diagnosis
D014408:Biomarkers, Tumor; D002116:Calcitonin; D013007:Growth Hormone-Releasing Hormone