Surgical management of MEN-1 and -2: state of the art.

Abstract

Multiple endocrine neoplasia syndrome type 1 (MEN-1) consists of endocrine tumors of the parathyroid, the endocrine pancreas-duodenum, and the pituitary. Surveillance and screening for the endocrinopathies is recommended in gene carriers. Surgery for MEN-1-related hyperparathyroidism is generally performed as radical subtotal parathyroidectomy, because less surgery is likely to result in persistent or recurrent disease. Multiple endocrine neoplasia syndrome type 2 (MEN-2) consists of medullary thyroid carcinoma, pheochromocytoma, and hyperparathyroidism. Prophylactic thyroidectomy based on DNA testing in the MEN-2 syndrome is considered one of the greater achievements in cancer treatment, because it may be performed before thyroid carcinoma development and provides cure for the patient.

• Authors: Göran Akerström, Peter Stålberg
• Journal: The Surgical clinics of North America (Surg Clin North Am) Vol. 89 Issue 5 Pg. 1047-68 (Oct 2009) ISSN: 1558-3171 [Electronic] United States
• PMID: 19836484 (Publication Type: Journal Article, Review)

Topics

• Adrenal Gland Neoplasms (blood, genetics, surgery)
• Carcinoma, Medullary (blood, genetics, surgery)
• Endocrine Surgical Procedures (methods)
• Genetic Testing
• Genotype
• Humans
• Hyperparathyroidism (blood, genetics, surgery)
• Multiple Endocrine Neoplasia Type 1 (blood, genetics, surgery)
• Multiple Endocrine Neoplasia Type 2a (blood, genetics, surgery)
• Parathyroidectomy
• Pheochromocytoma (blood, genetics, surgery)