Sachdev Y
AVSM AMC Centre and School, Lucknow.
Indian Pediatr. 1990 Jan;27(1):27-32.
Four children aged 3 months to 14 years presenting with multiple mucosal neuromatosis (MMN) and having medullary carcinoma of the thyroid (MCT) along with various other associated features are reported. All patient had screening test for pheochromocytoma. Thyroid gland and Neuromatous growths were subjected to histological examination for confirmatory diagnosis. All patients were subjected to total thyroidectomy with removal of regional lymph nodes. They were evaluated periodically for a variable post-operative period at 2 to 4 years. The first degree relatives were also screened. One of the four children had left sided pheochromocytoma which responded satisfactorily to the adrenalectomy. Prophylactic total thyroidectomy with annual/biannual critical review to detect recurrence is advocated. It is recommended that first degree relatives of familial MMN Syndrome should also be subjected to annual screening in view of its autosomonal dominant transmission.
报告了4例年龄在3个月至14岁之间的儿童，他们患有多发性黏膜神经瘤病（MMN），伴有甲状腺髓样癌（MCT）以及各种其他相关特征。所有患者均接受了嗜铬细胞瘤筛查。对甲状腺和神经瘤样生长物进行组织学检查以确诊。所有患者均接受了全甲状腺切除术及区域淋巴结清扫。在术后2至4年的不同时间段对他们进行定期评估。对一级亲属也进行了筛查。4名儿童中有1名患有左侧嗜铬细胞瘤，肾上腺切除术后反应良好。提倡进行预防性全甲状腺切除术，并每年/每两年进行严格复查以检测复发情况。鉴于其常染色体显性遗传，建议家族性MMN综合征的一级亲属也应每年进行筛查。
