Type i multiple endocrine neoplasia--Wermer syndrome
Schmidt, C.; Schumacher, S.; Siewert, B.; Klingmüller, D.; Vogel, J.
Schweizerische Rundschau für Medizin Praxis 78(35): 935-940
1989
ISSN/ISBN: 1013-2058 PMID: 2572044 Document Number: 344996
Multiple endocrine neoplastic diseases are genetically determined conditions with particular organ patterns for endocrine tumors. In Type I or Wermer's syndrome the endocrine pancreas, anterior pituitary and parathyroids are involved, insulinoma being the most frequent pancreatic tumor. To facilitate diagnosis, a prolonged oral glucose tolerance test, a fasting test and determination of the glucose-insulin ratio are recommended. Localisation is sought by computer tomography and angiography. A gastrinoma is excluded on the basis of normal gastrin levels in serum and by means of the secretin-provocation-test. Pituitary tumors can be classified more closely with prolactin levels and releasing-hormone tests (LH-RH and TRH). Prolactinoma is the most frequent pituitary tumor and amenable to bromocryptin treatment. If Wermer's syndrome is suspected, primary hyperparathyroidism has to be excluded on the basis of calcium and parathormone levels. Chief cell hyperplasia or multiple adenomas are frequent. Surgical resection is necessary.