Abstract

ObjectivesUnlike 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.MethodsA 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.ResultsA 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.ConclusionOur 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.

Highlights

  • Cutaneous melanoma in children and adolescents is rare, with an incidence ranging between 0.3 and 1 per 100 000 children a year, and only a small percentage occurring before puberty [1,2,3,4]

  • We evaluated the clinical characteristics and outcomes of all pediatric and adolescent malignant melanoma patients managed at pediatric oncology centers in Australia and New Zealand over the past two decades

  • We report here a retrospective case series of all malignant melanomas occurring in children and adolescents in Australia and New Zealand from the ANZCHOG, who were managed at tertiary pediatric oncology centers over two decades

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Summary

Introduction

Cutaneous melanoma in children and adolescents is rare, with an incidence ranging between 0.3 and 1 per 100 000 children a year, and only a small percentage occurring before puberty [1,2,3,4]. Given that certain geographical areas of Australia and New Zealand have been reported to have the highest rates of adult melanoma in the world, it is important to review pediatric data and evaluate specific factors that influence prognosis and overall survival [7,8,9]. Histopathological diagnosis is complicated due to the similar histological appearance of malignant melanoma with more benign lesions in childhood, such as spitz nevi, atypical spitz nevi and the concept of melanocytic tumors of uncertain prognosis (MELTUMP). Molecular diagnostic tools, such as fluorescent in situ hybridization and genomic testing, are assisting pathologists to distinguish between these different entities [11, 12]

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