Different outcomes in sporadic versus familial medullary thyroid cancer
Different outcomes in sporadic versus familial medullary thyroid cancer
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DOI：
10.1002/hed.25463
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发表时间：
2019-01-01
影响因子：
2.9
通讯作者：
Alevizaki, Maria
中科院分区：
文献类型：
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作者：
Saltiki, Katerina;Simeakis, George;Alevizaki, Maria
背景甲状腺髓样癌（MTC）的临床病程各不相同，家族性病例（fMTC）的诊断早于散发性MTC（spMTC）。方法对273例MTCs进行1-35年（中位5.0年）随访，其中家族性MTCs 110例（40.3%），男性MTCs 38.5%。51例家族性病例因基因筛查阳性而手术。记录诊断和随访时的疾病程度。结果：诊断时的平均年龄为：fMTC = 33.85 ± 16.5岁（范围4-74）和spMTC = 52.6 ± 14.0岁（范围16-81，P <0.001）。当排除遗传筛查病例时，这种差异仍然存在。fMTCs更常为多灶性，体积更小，诊断时分期更有利（I期和II期：60.9% vs 47.9%，III期：30.0% vs 23.9%，IV期：9.1% vs 28.9%，P = 0.01）。fMTC术前和术后降钙素水平较低，缓解率较高（59.1% vs 47.2%），疾病进展率较低（8.2% vs 35.0%，P <0.001）。排除遗传筛查病例后，未观察到诊断时的分期差异。fMTC的预后比散发性更好（P = 0.002）; fMTC和spMTC之间10年无疾病进展的概率差异显著（86.4% vs 65.0%，P <0.001）。结论在排除遗传筛查病例后，尽管诊断时的分期相似，但与fMTCs相比，散发性疾病的预后仍然更差。
Background Medullary thyroid carcinoma (MTC) has varying clinical course with familial cases (fMTC) diagnosed earlier than sporadic MTC (spMTC). Methods A total of 273 MTCs (familial: n = 110 [40.3%], males: 38.5%) were followed for 1-35 years (median 5.0 years). Fifty one of the familial cases were operated because of positive findings at genetic screening. Disease extent at diagnosis and follow-up was recorded. Results Mean age at diagnosis was: fMTC = 33.85 +/- 16.5 years (range 4-74) and spMTC = 52.6 +/- 14.0 years (range 16-81, P < .001). This difference remained when genetic screening cases were excluded. fMTCs had more frequently multifocality, smaller size, and more favorable stage at diagnosis (stages I and II: 60.9% vs 47.9%, stage III: 30.0% vs 23.9%, stage IV: 9.1% vs 28.9%, P = .01). fMTC had lower preoperative and postoperative calcitonin, more frequently remission (59.1% vs 47.2%) and less frequently progressive disease (8.2% vs 35.0%, P < .001). After excluding genetic screening cases, no difference in stage at diagnosis was observed. Outcome was more favorable in fMTC compared to sporadic (P = .002); the 10-year probability of lack of progression of disease differed significantly between fMTCs and spMTCs (86.4% vs 65.0%, P < .001). Conclusion After excluding genetic screening cases, although stage at diagnosis is similar, disease outcome remains worse in sporadic compared to fMTCs.