[The clinical characteristics and therapy of multiple endocrine neoplasia type 2].

OBJECTIVE

To study the clinical characteristics and treatment of multiple endocrine neoplasia (MEN) type 2.

METHODS

The clinical features, diagnosis and treatment of from 1980 to 2002 8 cases of multiple endocrine neoplasia type 2 from Peking Union Medical College Hospital were reviewed and analyzed in clinical features, diagnosis and treatment retrospectively.

RESULTS

Seven cases were with paroxysmal hypertension, the highest blood pressure was over 200 mm Hg (1 mm Hg = 0.133 kPa). Tumor was found in 3 cases. The diagnosis was confirmed by B-ultrasonography, CT and urine catecholamine test: six cases with MEN 2a 2 with MEN 2b. Six cases were medullary carcinoma of thyroid with or without parathyroidoma or hyperplasia, 1 with multiple mucosal neuromata. One case was pheochromocytoma with marfan's syndrome; 7 cases were with bilateral adrenal tumor. Total resection or resection ectomy was performed on different stages on adrenal gland, parathyroid tubercle. Average follow-up was 9 years. And the feedback was good.

CONCLUSION

The diagnosis of multiple endocrine neoplasia type 2 depends on endocrine biochemical tests, B-ultrasonography and CT. When the pheochromocytoma and the other tumor exists at the same time, operation is the primary treatment, and it is better to remove the pheochromocytoma at the first.