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Wermer's syndrome

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An inherited genetic defect that produces hyperplasia or malignant tumours in several endocrine glands.

Description

An inherited genetic defect that produces hyperplasia or malignant tumours in several endocrine glands. This group of diseases had been classed according to the glands defect, MEN I, MEN II, and MEN III. Wermer’s syndrome is MEN 1: Tumours of the parathyroids, pancreatic islets cells, adrenal cortex, and thyroid.

Wermer's syndrome is the sum of the effects of multiple tumours in various organs occurring at the same time or consecutively. It is commonly described as a neoplastic disease, characterized by tumours or hyperplasia of the parathyroid and pituitary glands and the islands of Langerhans with increased incidence of adrenocortical and thyroid disease, the parathyroid gland being most severely affected. Other tumours, including bronchial, gastrointestinal, and mediastinal carcinoids, multiple soft tissue lipomas, and renal cortical neoplasms, are frequently associated. Peptic ulcer with gastric hypersecretion is a common feature of this syndrome. Chromophobic tumours, eosinophilic adenomas with acromegaly, and prolactin-secreting tumour may also occur. Zollinger-Ellison syndrome is also common and the disorder has been described as Zollinger-Ellison syndrome with additional endocrine and glandular disorders.

Both sexes affected with same frequency, and onset at all ages after first decade. Protean symptomatology according to glands involved and function of adenomas. Inheritance is autosomal dominant trait with complete penetrance and variable expressivity.

Bibliography

  • Jakob Erdheim (1874-1937):
    Zur normalen und pathologischen Histologie der Glandula thyroidea, parathyroidea und Hypophysis.
    Beiträge zur pathologischen Anatomie und zur allgemeinen Pathologie, 1903, 33: 158-236.
  • P. Wermer:
    Genetic aspect of adenomatosis of endocrine glands.
    American Journal of Medicine, New York, 1954, 16: 363-371.
  • D. J. Lulu, T. E. Corcoran, M. Andre:
    Familial endocrine adenomatosis with associated Zollinger-Ellison syndrome. Wermer's syndrome.
    The American Journal of Surgery, New York, May 1968, 115 (5): 695-701.

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