MEN 1
Epidemiology and Etiology:
- Autosomal dominant
- 10% are de novo
germline mutations
- very high
penetrance
- M=F
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Common sites:
- Parathyroids
- Hyperparathyroidism
(NOT parathyroid adenoma or parathyroid carcinoma) (85% by age 35)
- Pancreas
- Anterior pituitary
- Adrenal cortex
- Thymus
- Bronchi
- Uncommon:
Gross features:
Histologic features:
- Parathyroids
- Note: parathyroid
adenoma should not be diagnosed in the setting of MEN 1 regardless of
the histologic findings
Immunophenotype:
Marker:
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Sensitivity:
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Specificity:
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Molecular features:
Other features:
- majority are
asymptomatic in terms of hyperparathyroidism
References:
- WHO Tumours
of Endocrine Organs (2004)