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The clinical features and treatment of pheochromocytoma in multiple endocrine neoplasia type 2a (MEN2A) patients

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Author:
No author available
Journal Title:
CHINA CLINICAL PRACTICAL MEDICINE
Issue:
7
DOI:
10.3760/cma.j.issn1673-8799.2009.07.01
Key Word:
多发性内分泌腺瘤2A型;嗜铬细胞瘤;Multiple endocrine neoplasia type 2A;Pheochromocytoma

Abstract: Objective To investigate the clinical features and treatment methods of pheochromotocyto-ma in multiple endocrine neoplasia type 2A patients. Methods Three unrelated families, among of which 8 were clinically diagnosed MEN2A patients, pheochromocytoma was found in all( 100% )of the 8 MEN2A patients. We analysis the clinical feature of blood pressure, the result of 24 hour urine vanillyl mandelic acid (VMA) and adre-nal CT, the treatment methods of pheochromocytoma. Results Medullary thyroid carcinoma(MTC) was found in 7(87.5% )patients,and pheochromocytoma was found in all(100%)of the 8 MEN2A patients. Hyperplasia or adenoma of the parathyroid gland was not found in any of these cases. Among them, pheochromocytoma was the initial demonstration of the syndrome in 6 patients(75%) ,and bilateral phoochromocytomas were found in 7 of them (87.5 %). 3 (37.5 %)patients present as sustained hypertension and 5 (62.5 %)patients present as parox-ysmal hypertension,75% of VMA was elevated and another 25% VMA/Cr was elevated. 4 (50%) patients were operated by open method and 4 (50%) patients were operated by video-assisted laparoscopic method. Conclu-sion The study indicated that pheochromocytomns was usually bilateral in MEN2A patients. The hypertension may be sustained or paroxysmal, the examinations of biology, imaging and RET gene mutation were useful for early diagnosis and treatment of pheochromotocytoma in MEN2A patients. Laparoscopic surgery was the best choice of treatment.

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