Management of hyperparathyroidism (PHP) in MEN2 syndromes in Europe
- PMID: 23514325
- PMCID: PMC3599702
- DOI: 10.1186/1756-6614-6-S1-S10
Management of hyperparathyroidism (PHP) in MEN2 syndromes in Europe
Abstract
Hyperparathyroidism occurs in 20-30% of MEN2A syndrome patients. It is usually associated with mild disease and is frequently asymptomatic, especially in younger age. There is genotype/phenotype association and PHP is usually associated with codon 634 mutations; however association with more "rare" mutations has also been reported. The pathology of the parathyroid glands includes hyperplasia, adenoma or a combination of the two. The optimal surgical management of this entity has not been defined yet.
References
-
- Schuffenecker I, Virally-Monod M, Brohet R, Goldgar D, Conte-Devolx B, Leclerc L, Chabre O, Boneu A, Caron J, Houdent C, Modigliani E, Rohmer V, Schlumberger M, Eng C, Guillausseau PJ, Lenoir GM. Risk and penetrance of primary hyperparathyroidism in multiple endocrine neoplasia type 2A families with mutations at codon 634 of the RET proto-oncogene. Groupe D'etude des Tumeurs à Calcitonine. J Clin Endocrinol Metab. 1998;83:487–491. doi: 10.1210/jc.83.2.487. - DOI - PubMed
-
- Kraimps JL, Denizot A, Carnaille B, Henry JF, Proye C, Bacourt F, Sarfati E, Dupond JL, Maes B, Travagli JP, Boneu A, Roger P, Houdent C, Barbier J, Modigliani E. Primary hyperparathyroidism in multiple endocrine neoplasia type IIa: retrospective French multicentric study. Groupe d'Etude des Tumeurs á Calcitonine (GETC, French Calcitonin Tumors Study Group), French Association of Endocrine Surgeons. World J Surg. 1996;20:808–812. doi: 10.1007/s002689900123. - DOI - PubMed
-
- Raue F, Frank-Raue K. Genotype-phenotype relationship in multiple endocrine neoplasia type 2. Implications for clinical management. Hormones (Athens) 2009;8:23–28. - PubMed
LinkOut - more resources
Full Text Sources
Other Literature Sources
