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Management of Multiple Endocrine Neoplasia of type 2a (MEN2a) without RET gene: about a case and review of the literature.

Management of MEN2a is conditioned by the mutation of the RET gene. Aim of this study is to discuss the diagnostic and surgical strategy. Case report: seventy-year-old woman is admitted for hyperparathyroidism. Examination found a good general condi-tion and a nodular goiter. Biology found a Medullary Carcinoma of the Thyroid (MTC) and a pheochromocytoma. It was a MEN2a. Left adrenalectomy was performed, followed by right thyroidectomy with lymphadenectomy and parathyroidectomy. Pathological examination of the surgical specimens found a pheochromocytoma, a MTC and a parathyroid hyperplasia. Genetic analysis did not find a mutation in the RET gene. Conclusion: management strategy relies mainly on the existence of MTC, which is the main prognostic factor. Absence of mutation of the RET gene in the index case is a rare situation and can modify the ma-nagement.


Auteur(s) : M. Seck, E. Housseau
Pages : 86-88
Année de publication : 2017
Revue : Surg Chron 2017;
N° de volume : 22(2)
Type : Article
Mise en ligne par : SECK Mamadou