多发性内分泌腺瘤ⅡA型的超声表现

目的 探讨多发性内分泌腺瘤ⅡA型的超声表现,提高对该病的认识和诊断水平.方法 回顾性分析经临床手术病理证实的11例患者的超声及临床资料.结果 11例患者分属4个家系,尿3-甲氧-4羟基杏仁酸（VMA）均升高.甲状腺髓样癌发病均早于肾上腺嗜铬细胞瘤,超声声像图示：病灶多侵及甲状腺双叶（8/11）,以低回声为主（15/19）,并伴有钙化灶（18/19）,其中粗大钙化灶居多（12/18）,边界多见不规则声晕（11/19）,病灶血流多为Ⅲ级（15/19）.伴颈部淋巴结转移者8例（8/11）;病理示其中有5例（5/11）合并甲状旁腺增生,超声确诊其中4例.肾上腺嗜铬细胞瘤定位诊断符合率为90.90％ （20/22）,定性诊断符合率为90％（18/20）.结论 超声对该病的定位定性诊断具有重要价值,可作为对该病家族成员随访的首选影像学检查方法.

Ultrasonic appearance of multiple endocrine neoplasia type Ⅱ A

Objective To explore the ultrasonographic findings of the multiple endocrine neoplasia type H A and to improve the understanding and diagnostic level of this disease. Methods We retrospectively analyzed the ul- trasonographic findings and clinical data of 11 patients of the multiple endocrine neoplasia type Ⅱ A confurmed by pa- thology. Results All 11 patients belonging to four families showed raised urinary 3-methoxy-4 hydroxy vanilly- mandelic acid （VMA） levels. The onset of medullary thyroid carcinoma was earlier than adrenal pheochromocyto- ma. The ultrasonographic findings were as follows： lesion invades bilateral lobes of thyroid （8/11）, mainly with hy- poecho （15/19）, accompanied by calcifications （18/19）, the majority of which were bulky calcifications （12/18）, ir- regular border acoustic halo mostly （11/19）, lesion blood flow of grade Ⅲ （15/19）. 8 cases had cervical lymph node metastasis （8/11）. 5 cases （5/11） combined with parathyroid hyperplasia, in which 4 cases were diagnosed by the ul- trasound. The coincidence rate of localization and qualitative diagnosis of adrenal pheochromocytoma was 90.90% （20/22） and 90% （18/20） respectively. Conclusion Ultrasound has important value on the localization and qualita- tive diagnosis of this disease, and is the first choice of the follow-up imaging examination for members of this dis- ease family.