Surgical management of MEN-1 and -2: state of the art.
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.
Akerström
Göran
G
Department of Surgery, University Hospital, Uppsala, 751 85 Sweden. goran.akerstrom@surgsci.uu.se
Stålberg
Peter
P
D000310:Adrenal Gland Neoplasms / Q000097:blood / Q000235:genetics / Q000601:surgery; D018276:Carcinoma, Medullary / Q000097:blood / Q000235:genetics / Q000601:surgery; D013507:Endocrine Surgical Procedures / Q000379:methods*; D005820:Genetic Testing; D005838:Genotype; D006801:Humans; D006961:Hyperparathyroidism / Q000097:blood / Q000235:genetics / Q000601:surgery; D018761:Multiple Endocrine Neoplasia Type 1 / Q000097:blood / Q000235:genetics / Q000601:surgery*; D018813:Multiple Endocrine Neoplasia Type 2a / Q000097:blood / Q000235:genetics / Q000601:surgery*; D016105:Parathyroidectomy; D010673:Pheochromocytoma / Q000097:blood / Q000235:genetics / Q000601:surgery