Clinical Analysis Of Multiple Endocrine Neoplasia Type Ⅱ

Objective:To improve diagnosis effectiveness for patients with multiple endocrine neoplasia typeⅡ。Focus on①the genetics and clinical features of MEN-Ⅱ;②the clinical manifestations,diagnostic endocrinology,diagnostic imaging and genetics of MEN-Ⅱ;③MEN-Ⅱof pheochromocytoma and medullary thyroid carcinoma(MTC) surgery preoperative preparation and intraoperative handling.Methods:36 patients with MEN-Ⅱwere reviewed and presented between 2000 to 2008 in this paper。The clinical features,the diagnosis,principle of treatment and prognosis were discussed。Results:1.In all of 36 cases,13 patients were male and 23 were female,the average age was 35 years old,the average of the course was 5.6 years。In which 31 cases of MEN-ⅡA, 12 cases of male,19 cases of female,and the average age was 36;5 cases of MEN -ⅡB and the average age was 29.4。2.22 cases of positive family history。3.There are 36 cases found 14 cases of thyroid nodules and pheochromocytoma。Bilateral involvement of the thyroid gland in 31 cases,4 cases of unilateral thyroid。2 cases of parathyroid hormone。21 cases of bilateral adrenal glands,14 cases of unilateral adrenal gland,which next to the abdominal aorta complicated by pheochromocytoma in 1 case.。Skin,tongue mucosa of five cases of neurofibroma, which complicated breast fibroma,one cases of uterine fibroids。At the same time, involving 14 cases of thyroid and adrenal glands,。4.Anesthesia for 19 cases of clear-cut,18 cases of general anesthesia,one case of epidural anesthesia。5.Follow-up of 36 cases in 33 cases,two more than 13 cases of surgical history; pheochromocytoma were 4 cases of recurrence;medullary thyroid cancer recurrence in 3 cases,2 cases of death。Conclusions:1.The cases in female were more than male,but the age of onset were much the same。2.MEN-Ⅱmore than the incidence in young people,of which incidence of MEN-ⅡB than type MEN-ⅡA type earlier。3.MEN-Ⅱof the medullary thyroid carcinoma and pheochromocytoma mostly are bilateral multi-focal disease,and pheochromocytoma relatively has late age of onset。4.Both pheochromocytoma or medullary thyroid carcinoma(MTC),general anesthesia has advocated in the surgery of MEN-Ⅱ。5.For medullary thyroid carcinoma(MTC),early diagnosis and early radical surgery,that is,thyroid resection and dissection of cervical lymph node sections, can significantly improve the prognosis,and even to cure。6.When the medullary thyroid carcinoma(MTC) and phenochromocytoma exist at the same time,it better to do the excision of phenochromocytoma firstly。7.The first degree relative of MEN-Ⅱpatient require screening。Earlydiagnosis and treatment can improve prognosis obviously。...