Table 1.

Tissue selectivity of tumours in probands and families with MEN1

Hormone-secreting tumours
Parathyroid adenoma (90%)	Anterior pituitary tumour
Pancreaticoduodenal tumour	Prolactinoma (20%)
Gastrinoma (40%)	Other growth hormone + prolactin (5%)
Insulinoma (10%)	Growth hormone (5%), NF (5%)
NF, also pancreatic polypeptide (20%)	ACTH (2%) or TSH (rare)
Glucagon, vasoactive intestinal polypeptide,	Adrenal
somatostatin, etc. (2%)	Cortex NF (25%)
Foregut carcinoid	Medulla (1%)
Thymic carcinoid NF (4%)
Bronchial carcinoid NF (2%)
Gastric enterochromaffin-like NF (10%)
Hormone non-secreting tumours
Facial angiofibroma (85%)	Leiomyoma
Truncal collagenoma (70%)	Uterus in female (30%?)
Lipoma (30%)	Oesophagus (5%)
Meningioma (5%)	Ependymoma (1%)
Barrett’s oesophagus (5%)

NF = Non-functioning; TSH = thyroid-stimulating hormone. Percentages in parentheses/brackets indicate penetrance at age 40 years. Terms in bold font indicate tumour type with malignant potential for 25% or more of cases. Modified from Gagel and Marx [2].