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儿童和青少年皮肤黑色素瘤:意大利儿科罕见肿瘤项目经验。

Cutaneous melanoma in children and adolescents: the Italian rare tumors in pediatric age project experience.

机构信息

Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy.

Hematology-Oncology Division, Department of Pediatrics, Padova University Hospital, Padova, Italy.

出版信息

J Pediatr. 2014 Feb;164(2):376-82.e1-2. doi: 10.1016/j.jpeds.2013.10.012. Epub 2013 Nov 16.

Abstract

OBJECTIVE

To describe a series of cutaneous melanoma in children collected by the Italian Rare Tumors in Pediatric Age project.

STUDY DESIGN

From 2000 to 2012, 54 patients younger than 18 years of age were prospectively registered and treated at 12 Italian pediatric centers on the basis of the same diagnostic/therapeutic recommendations and with the same forms to record clinical data.

RESULTS

Considering the estimated annual incidence in Italy, the registered cases accounted for 30% of those expected in children and 10% of adolescents. Clinically, 47% of the tumors were amelanotic and 81% were raised, 39% of cases had tumor thickness >2 mm, and 36% had lymph node involvement. For the whole series, 5-year event-free survival and overall survival rates were 75.2% and 84.6%, respectively. Patient survival correlated with tumor stage and ulceration. No relapses were recorded for T1-2 (thickness <2 mm), N0, and stage 0-I-II cases.

CONCLUSION

We suggest that the variables influencing survival in children with melanoma are the same as for adults, the clinical approach used in adults is feasible in children, and pediatric cases are more likely to have advanced disease at diagnosis but similar survival. New effective drugs are needed for advanced disease, and biological studies and international cooperative schemes are warranted.

摘要

目的

描述由意大利儿科罕见肿瘤项目收集的一系列儿童皮肤黑色素瘤病例。

研究设计

2000 年至 2012 年,12 家意大利儿科中心前瞻性登记并治疗了 54 名年龄小于 18 岁的患者,这些患者采用相同的诊断/治疗建议,并使用相同的表格记录临床数据。

结果

考虑到意大利的预估年发病率,登记病例占儿童预期病例的 30%,占青少年的 10%。临床方面,47%的肿瘤为无色素性,81%为隆起性,39%的病例肿瘤厚度>2mm,36%的病例有淋巴结受累。在整个系列中,5 年无事件生存率和总生存率分别为 75.2%和 84.6%。患者生存率与肿瘤分期和溃疡有关。T1-2(厚度<2mm)、N0 和 0-I-II 期的病例无复发记录。

结论

我们认为影响儿童黑色素瘤患者生存的因素与成人相同,成人中使用的临床方法在儿童中是可行的,且儿科病例在诊断时更有可能处于晚期疾病,但生存率相似。需要新的有效药物治疗晚期疾病,需要进行生物学研究和国际合作计划。

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