Primary Hyperparathyroidism in Patients with Multiple Endocrine Neoplasia Type 1

Table 1

Tumours associated with MEN-1 and their penetrance.

Localization	Clinical manifestation	Penetrance
Endocrine
Parathyroid	Primary hyperparathyroidism (bone demineralisation, kidney stones)	90%
Enteropancreatic
Gastrinoma	Zollinger-Ellison syndrome (severe peptic ulceration)	40%
Insulinoma	Recurrent neuroglycopenia	10%
Nonfunctioning	Late diagnosis (symptoms related to tumor mass)	20%
Other	WDHA syndrome, diabetes mellitus	2%
Pituitary gland
Prolactinoma	Galactorrhea, menstrual period anomalies, reduced libido, erectile dysfunction, infertility	20%
Other	Cushing syndrome, pituitary gland insufficiency	17%
Adrenal
Nonfunctioning cortex	“Incidentaloma”	20%
Pheochromocytoma	Paroxysmal or permanent arterial hypertension, paroxysmal tachyarrhythmia, diabetes mellitus	<1%
Foregut neuroendocrine tumours
Gastric	Carcinoid syndrome	10%
Thymic	Carcinoid syndrome	2%
Bronchial	Carcinoid syndrome, chronic cough	2%
Nonendocrine
Facial angiofibromas	Local symptoms related to tumor mass	85%
Collagenomas	Local symptoms related to tumor mass	70%
Lipomas	Local symptoms related to tumor mass	30%
Leiomyomas	Local symptoms related to tumor mass	10%
Meningiomas	Intracranial pressure related symptoms	5%
Ependymomas	Local symptoms related to tumor mass	1%