Girelli M E, Nacamulli D, Pelizzo M R, De Vido D, Mian C, Piccolo M, Busnardo B
Istituto di Semeiotica Medica, Università di Padova, Italy.
Thyroid. 1998 Jun;8(6):517-23. doi: 10.1089/thy.1998.8.517.
This article describes the findings of a retrospective analysis of data obtained on 78 patients with medullary thyroid carcinoma (MTC), recorded between 1969 and 1986, and then followed at the Thyroid Center of Padua (Italy). The ages of the patients ranged between 15 and 89 years, with a median age of 45. The female to male (F:M) ratio was 2.9:1. All patients except 2 had total thyroidectomy. In 70 cases the tumor was of sporadic type; there were 3 familial non-multiple endocrine neoplasia (MEN) MTC; 3 MEN IIa; and 2 MEN IIb. The median duration of follow-up was 15.9 years (13 patients were followed up between 15 and 20 years, and 9 longer than 20). At diagnosis, the tumor was intrathyroid (stage I and II) in 31 patients, with local lymphnodes involved (stage III) in 41 patients, and with distant metastases (stage IV) in 6 patients. A total of 34 patients died (4 were at stage II at diagnosis, 26 at stage III, and 4 at stage IV); 4 of them died of unrelated causes, the others with tumor. The median survival rate of the deceased patients was 6 years (41% of these patients died within 3 years and 24% after more than 10 years); 76% of the deceased patients were older than 45 years at diagnosis. A total of 44 patients are still alive, 22 are alive free of disease (with follow-up between 10 and 24 years, median 14.2 years) and 22 are alive with disease (median follow-up 12.2 years). Only 30% of the patients of both these groups was older than 45 years at diagnosis. Survival is strongly related to tumor stage and to age at diagnosis, because only 8 of the 34 deceased patients were younger than 45 years (and 2 of them died of unrelated causes); moreover, patients who were treated at earlier stages of the disease had better prognosis. Survival rate at 10 and 20 years was 95% for patients with tumor limited to the thyroid, whereas it was 55% and 28.6%, respectively, for patients at stage III and IV. Bone metastases were correlated with worse prognosis than distant metastases only to soft tissues. The sex did not affect survival. None of the patients who had postoperative low serum calcitonin (CT) levels and no response to pentagastrin stimulation showed recurrences in the follow-up. In patients with postoperative elevated serum CT levels, recurrences of the tumor increased over time. However, 30% of these patients continued to show only elevated CT levels without evidence of the disease, even after 15 years.
本文描述了一项回顾性分析的结果，该分析数据来自于1969年至1986年间记录的78例甲状腺髓样癌（MTC）患者，这些患者随后在意大利帕多瓦甲状腺中心接受随访。患者年龄在15至89岁之间，中位年龄为45岁。女性与男性（F:M）比例为2.9:1。除2例患者外，所有患者均接受了甲状腺全切除术。70例患者的肿瘤为散发性；有3例家族性非多发性内分泌肿瘤（MEN）MTC；3例MEN IIa；2例MEN IIb。中位随访时间为15.9年（13例患者随访15至20年，9例患者随访超过20年）。诊断时，31例患者的肿瘤位于甲状腺内（I期和II期），41例患者有局部淋巴结受累（III期），6例患者有远处转移（IV期）。共有34例患者死亡（4例诊断时为II期，26例为III期，4例为IV期）；其中4例死于无关原因，其他患者死于肿瘤。死亡患者的中位生存率为6年（这些患者中41%在3年内死亡，24%在10年以上死亡）；76%的死亡患者诊断时年龄大于45岁。共有44例患者仍存活，22例无疾病存活（随访10至24年，中位14.2年），22例带瘤存活（中位随访12.2年）。这两组患者中只有30%诊断时年龄大于45岁。生存率与肿瘤分期和诊断时年龄密切相关，因为34例死亡患者中只有8例年龄小于45岁（其中2例死于无关原因）；此外，疾病早期接受治疗的患者预后较好。肿瘤局限于甲状腺的患者10年和20年生存率为95%，而III期和IV期患者分别为55%和28.6%。骨转移比仅软组织远处转移的预后更差。性别不影响生存率。术后血清降钙素（CT）水平低且对五肽胃泌素刺激无反应的患者在随访中均未出现复发。术后血清CT水平升高的患者，肿瘤复发随时间增加。然而，这些患者中有30%即使在15年后仍仅表现为CT水平升高而无疾病证据。
