Demeter J G, De Jong S A, Lawrence A M, Paloyan E
Department of Surgery, Loyola University Stritch School of Medicine, Maywood, Ill.
Surgery. 1991 Dec;110(6):956-61; discussion 961-3.
Anaplastic thyroid carcinoma, in contrast to well-differentiated thyroid carcinoma, has a dismal prognosis, and little progress has been made in improving survival for this disease. We reviewed our experience during a 23-year period to identify risk factors and possible methods to improve outcome. Between 1966 and 1989, 340 patients with thyroid carcinoma underwent operation. Of these, 17 (5%) were undergoing operative treatment of anaplastic or undifferentiated thyroid carcinoma. The female/male ratio was 3.5:1, and mean age at presentation was 63 years. The most common presenting symptoms included neck mass, voice change, or dysphagia. Unusual presentations included symptomatic bradycardia from compression of the vagus nerve and superior vena cava syndrome. Four patients had a history of well-differentiated thyroid carcinoma. Nine patients had been diagnosed or treated in the past for "goiter" or a neck mass, and four patients had concurrent differentiated thyroid carcinoma associated with the anaplastic tumor. Thus 13 (76%) of 17 patients had a previous thyroid disorder, benign or differentiated malignant, and eight (47%) of 17 patients had previous or concurrent differentiated thyroid carcinoma. At the time of presentation, six patients had unilateral true vocal cord paralysis. At operation, 14 patients had local extension of the tumor and four required tracheostomy. Only five of 12 patients showed response to postoperative radiation therapy. Overall median survival was 12 months, and 13 (76%) of 17 patients died. The two patients alive longer than 12 months had only small foci of anaplastic carcinoma in association with well-differentiated carcinoma. Anaplastic thyroid carcinoma is a locally and systemically aggressive disease, with long-term survival seen only in those with well-localized anaplastic tumor. The major risk factor in this series is a history of previous benign or malignant thyroid disease. Because of this, a more aggressive approach to thyroid masses may be warranted. Long-standing goiters or benign nodules should be followed carefully and considered for resection if they grow or do not respond to medical therapy, and total thyroidectomy for malignant disease may obviate the subsequent development of anaplastic carcinoma. This method of early diagnosis and resection of abnormal thyroid tissue seems to be the only method currently available to improve the nearly uniform fatality of this disease.
与分化良好的甲状腺癌相比，间变性甲状腺癌的预后很差，在改善这种疾病的生存率方面进展甚微。我们回顾了23年间的经验，以确定危险因素和可能改善预后的方法。1966年至1989年间，340例甲状腺癌患者接受了手术。其中，17例（5%）接受了间变性或未分化甲状腺癌的手术治疗。男女比例为3.5:1，就诊时的平均年龄为63岁。最常见的症状包括颈部肿块、声音改变或吞咽困难。不寻常的表现包括迷走神经受压引起的症状性心动过缓和上腔静脉综合征。4例患者有分化良好的甲状腺癌病史。9例患者过去曾被诊断或治疗过“甲状腺肿”或颈部肿块，4例患者同时患有与间变性肿瘤相关的分化型甲状腺癌。因此，17例患者中有13例（76%）既往有甲状腺疾病，良性或分化型恶性，17例患者中有8例（47%）既往或同时患有分化型甲状腺癌。就诊时，6例患者出现单侧真性声带麻痹。手术时，14例患者肿瘤有局部侵犯，4例需要气管切开术。12例患者中只有5例对术后放疗有反应。总体中位生存期为12个月，17例患者中有13例（76%）死亡。存活超过12个月的2例患者仅在分化良好的癌中伴有小灶间变性癌。间变性甲状腺癌是一种局部和全身侵袭性疾病，只有在间变性肿瘤局限的患者中才能见到长期生存。本系列中的主要危险因素是既往有良性或恶性甲状腺疾病史。因此，对于甲状腺肿块可能需要采取更积极的治疗方法。长期存在的甲状腺肿或良性结节应密切随访，如果它们生长或对药物治疗无反应则考虑切除，对于恶性疾病行全甲状腺切除术可能避免随后发生间变性癌。这种早期诊断和切除异常甲状腺组织的方法似乎是目前唯一可用于改善这种几乎一致致命性疾病的方法。
