Multiple endocrine neoplasia.
The multiple endocrine neoplasia (MEN) syndromes are well-defined disorders characterized by familial inheritance of specific endocrine tumors. The parathyroid, endocrine, pancreas, and pituitary tumors of MEN-1 are described by frequency and symptomatology. The effectiveness of surgery, symptomatic therapy, and panendocrine suppression by the somatostatin congener octreotide are discussed. Evidence indicates that the MEN-1 gene is located on chromosome 11 and tightly linked markers can help identify family members at risk for inheriting the gene. In MEN-2, the effectiveness of biochemical screening for thyroidal C-cell neoplasms and early thyroidectomy are described. New imaging techniques have been developed to identify medullary thyroid carcinoma and pheochromocytoma in MEN-2. Genetic analysis has identified markers on chromosome 10 closely linked to the MEN-2a gene, allowing better identification of family members likely to develop the syndrome.
Caruso
D R
DR
Ohio State University School of Medicine, Columbus.
O'Dorisio
T M
TM
Mazzaferri
E L
EL
D000310:Adrenal Gland Neoplasms; D014408:Biomarkers, Tumor; D002277:Carcinoma; D002879:Chromosomes, Human, Pair 10; D015342:DNA Probes; D006801:Humans; D006961:Hyperparathyroidism / Q000209:etiology; D006965:Hyperplasia; D009377:Multiple Endocrine Neoplasia / Q000150:complications / Q000175:diagnosis / Q000235:genetics; D010190:Pancreatic Neoplasms; D009384:Paraneoplastic Endocrine Syndromes / Q000209:etiology; D010673:Pheochromocytoma; D010911:Pituitary Neoplasms; D012150:Polymorphism, Restriction Fragment Length; D013961:Thyroid Gland / Q000473:pathology; D013964:Thyroid Neoplasms
D014408:Biomarkers, Tumor; D015342:DNA Probes