Trotoux J, Pinel J
Ann Otolaryngol Chir Cervicofac. 1979 Jan-Feb;96(1-2):65-86.
Medullary carcinoma of the thyroid develops in the parafollicular C cells, the origin of which is in the neural crest. It is characterized clinically by the presence of motor diarrhea, vasomotor attacks, and immunologically by the presence of an amyloid stroma and the appearance of an anaplastic carcinoma. It is frequently associated with a pheochromocytoma (Sipple's syndrome), parathyroid adenomas and cytaneo mucosal manifestations (phacomatosis, multiple mucosal neuromas). The C cells may secrete thyrocalcitonin and the estimation of the substance may be sometimes used in pre-operative diagnosis and, above all, subsequent observation and examination for the presence of metastases. Treatment is based upon surgery (total thyroidectomy) with lymphnode dissection. Complementary treatment (radiotherapy, chemotherapy, opotherapy) is given only when surgery is inedequate or recurrences occur.
甲状腺髓样癌起源于神经嵴的滤泡旁C细胞。其临床特征为运动性腹泻、血管舒缩发作,免疫特征为淀粉样基质以及未分化癌的出现。它常与嗜铬细胞瘤(西普尔综合征)、甲状旁腺腺瘤及皮肤黏膜表现(错构瘤、多发性黏膜神经瘤)相关。C细胞可分泌降钙素,该物质的测定有时可用于术前诊断,最重要的是用于后续观察及有无转移的检查。治疗以手术(甲状腺全切术)加淋巴结清扫为基础。仅在手术不充分或复发时才给予辅助治疗(放疗、化疗、靶向治疗)。