multiple endocrine neoplasia type IIa

Multiple endocrine neoplasia (MEN) type IIa, also known as Sipple syndrome, accounts for most cases of MEN2 and is characterized by:

• pheochromocytomas: in 50% of patients, often bilateral, and can be extra-adrenal
• medullary thyroid cancer: 100% of patients, aggressive, and may secrete calcitonin
• parathyroid hyperplasia: only seen in 20% of patients, and often presents with hypercalcemia and renal calculi

Mnemonic:

• PMP

Pathology

Genetics

A small proportion of individuals have a RET D631 proto-oncogene mutation.

See also

• MEN1 (Wermer syndrome)
• MEN2 (multiple endocrine adenomatosis)
  • MEN2a (Sipple syndrome)
  • MEN2b (mucosal neuroma syndrome)
  • familial medullary thyroid carcinoma
• MEN4
• Carney complex

Siehe auch:

• Nierensteine
• Phäochromozytom
• multiple endokrine Neoplasie Typ 1
• Medulläres Schilddrüsenkarzinom
• MEN IIb
• Multiple endokrine Neoplasien Typ 2

und weiter:

• primärer Hyperparathyreoidismus
• multiple endocrine neoplasia type IIb
• multiple endocrine neoplasia syndromes
• Neurocristopathien