Phéochromocytomes surrénaliens bilatéraux au cours de la maladie de von Hippel-Lindau
作者： D Touiti , B Seket , E Deligne , L Badet , M Colombel
DOI: 10.1016/S0003-4401(01)00053-5
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摘要: Resume Le pheochromocytome est une tumeur medullosurrenalienne developpee aux depens des cellules chromaffines. Elle survient dans 11 a 19 % cas de maladie von Hippel-Lindau (VHL), elle souvent bilaterale et la symptomatologie en fruste : l'hypertension arterielle isolee instable triade classique cephalee, palpitations, sueurs assez rarement observee. Nous rapportons quatre observations pheochromocytomes bilateraux chez patients porteurs d'une (trois phenotypes IIA un phenotype IIB). La etait au moment du diagnostic trois cas, le quatrieme patient, l'atteinte surrenale controlaterale survenue deux annees apres premiere surrenalectomie. Tous les ont eu surrenalectomie par chirurgie ouverte courte preparation 48 heures ; traitement substitutif ete instaure tous patients. morbidite faible, sont soumis surveillance prolongee pour depister d'eventuelles lesions VHL.
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