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儿童肿瘤中心治疗的儿童和青少年恶性黑色素瘤:一项澳大利亚和新西兰儿童肿瘤组(ANZCHOG)的研究。

Malignant Melanoma in Children and Adolescents Treated in Pediatric Oncology Centers: An Australian and New Zealand Children's Oncology Group (ANZCHOG) Study.

作者信息

Ryan Anne L, Burns Charlotte, Gupta Aditya K, Samarasekera Ruvishani, Ziegler David S, Kirby Maria L, Alvaro Frank, Downie Peter, Laughton Stephen J, Cross Siobhan, Hassall Timothy, McCowage Geoff B, Hansford Jordan R, Kotecha Rishi S, Gottardo Nicholas G

机构信息

Department of Haematology, Oncology and Bone Marrow Transplant, Perth Children's Hospital, Perth, WA, Australia.

Children's Cancer Centre, The Royal Children's Hospital, Melbourne, VIC, Australia.

出版信息

Front Oncol. 2021 Apr 29;11:660172. doi: 10.3389/fonc.2021.660172. eCollection 2021.

Abstract

OBJECTIVES

Unlike adults, malignant melanoma in children and adolescents is rare. In adult melanoma, significant progress in understanding tumor biology and new treatments, including targeted therapies and immunotherapy have markedly improved overall survival. In sharp contrast, there is a paucity of data on the biology and clinical behavior of pediatric melanoma. We report a national case series of all pediatric and adolescent malignant melanoma presenting to ANZCHOG Childhood Cancer Centers in Australia and New Zealand.

METHODS

A retrospective, descriptive, multi-center study was undertaken to identify patients less than 18 years of age treated for cutaneous malignant melanoma over a twenty-year period (1994 to 2014). Data on clinical characteristics, histopathology, and extent of disease, treatment and follow-up are described.

RESULTS

A total of 37 cases of malignant melanoma were identified from all of the Australasian tertiary Childhood Cancer Centers. The median age was 10 years (range 1 month - 17 years). Clinically, the most common type of lesion was pigmented, occurring in sixteen (57%) patients, whilst amelanotic was seen in 7 patients (25%). In 11 (27.9%) the Breslow thickness was greater than 4mm. A total of 11 (29.7%) patients relapsed and 90% of these died of disease. Five-year event free survival (EFS) and overall survival were 63.2 (95% CI: 40.6 - 79.1) and 67.7% (95% CI: 45.1 - 82.6) respectively.

CONCLUSION

Our data confirms that melanoma is a rare presentation of cancer to tertiary Australasian Childhood Cancer Centers with only 37 cases identified over two decades. Notably, melanoma managed in Childhood Cancer Centers is frequently at an advanced stage, with a high percentage of patients relapsing and the majority of these patients who relapsed died of disease. This study confirms previous clinical and prognostic information to support the early multidisciplinary management in Childhood Cancer Centers, in conjunction with expert adult melanoma centers, of this rare and challenging patient group.

摘要

目的

与成人不同,儿童和青少年恶性黑色素瘤较为罕见。在成人黑色素瘤中,对肿瘤生物学的理解以及包括靶向治疗和免疫治疗在内的新治疗方法取得了重大进展,显著提高了总体生存率。形成鲜明对比的是,关于儿童黑色素瘤生物学和临床行为的数据却很匮乏。我们报告了澳大利亚和新西兰ANZCHOG儿童癌症中心收治的所有儿童和青少年恶性黑色素瘤的全国病例系列。

方法

开展了一项回顾性、描述性、多中心研究,以确定在20年期间(1994年至2014年)接受皮肤恶性黑色素瘤治疗的18岁以下患者。描述了临床特征、组织病理学、疾病范围、治疗及随访数据。

结果

从所有澳大利亚和新西兰的三级儿童癌症中心共识别出37例恶性黑色素瘤病例。中位年龄为10岁(范围1个月至17岁)。临床上,最常见的病变类型是色素沉着型,16例(57%)患者出现此类型,而无色素型见于7例(25%)患者。11例(27.9%)患者的Breslow厚度大于4mm。共有11例(29.7%)患者复发,其中90%死于疾病。5年无事件生存率(EFS)和总生存率分别为63.2%(95%CI:40.6 - 79.1)和67.7%(95%CI:45.1 - 82.6)。

结论

我们的数据证实,黑色素瘤在澳大利亚和新西兰的三级儿童癌症中心是一种罕见的癌症表现形式,二十年间仅识别出37例。值得注意的是,在儿童癌症中心接受治疗的黑色素瘤患者通常处于晚期,复发率很高,且大多数复发患者死于疾病。本研究证实了先前的临床和预后信息,以支持儿童癌症中心与成人黑色素瘤专家中心联合,对这一罕见且具有挑战性的患者群体进行早期多学科管理。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b7af/8117414/b901ce3f33f4/fonc-11-660172-g001.jpg

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