Cutaneous malignant melanoma in children and adolescents treated in pediatric oncology units

Abstract

Objectives: Recent progress in the understanding of tumor biology and new targeted therapies has led to improved survival in adults with malignant melanoma (MM). MM is rare in children, especially before puberty. We report here our experience with pediatric patients with MM, describe the clinical presentation, treatment and evolution, and compare prepubescent and postpubescent disease.Methods: A retrospective, descriptive, national multicenter study was undertaken of 52 cases of MM in children and adolescents. Demographic, histopathology, treatment evolution data, and survival distributions are described.Results: Median age was 15 years (5-18). The tumors were often amelanotic (45%) and raised (83%), and Breslow thickness was greater than 4 mm in 35% of cases. Histological examination showed superficial spreading (n = 16) or spitzoid (n = 16) or nodular (n = 9) pattern. Twelve children (23%) were less than 10 years of age. The spitzoid histotype was more frequent in prepubescent children (seven of 12). Seventeen patients relapsed, of whom four had skin lesions initially diagnosed as benign. Ten patients died after relapse. Five-year event-free survival and overall survival were 62.7% (95% confidence interval [CI]: 45.3-76) and 75.5% (95% CI: 56.8-87.1), respectively.Conclusions: MM appears to be different in prepubescent children, of whom most had a spitzoid histotype. Diagnosis can be difficult, leading to delay in treatment. New biological tools to identify targets for treatment in MM and to differentiate spitzoid melanomas from Spitz nevi now exist. As effective targeted therapies are now available, we recommend requesting biological examination of all melanocyte-derived skin lesions in children that could be malignant.

Pediatric patients with cutaneous melanoma: A European study

“…This study concerned patients prospectively registered under national cooperative projects dedicated to rare pediatric tumors in Italy (Tumori Rari in Età Pediatrica [TREP]) (n = 43), Poland (Polish Pediatric Rare Tumor Study Group, n = 23), Germany (Seltene Tumorerkrankungen in der Pädiatrie, n = 31), and France (Groupe FRAnCais Des TUmeurs Rares de l'Enfant [FRaCTurE], n = 43). Additional cases were collected from the prospective German Central Malignant Melanoma Registry (CMMR, n = 65) and from a dermatology registry in Israel (n = 14).…”

“…It is noteworthy that, according to the definition adopted by pediatric cooperative groups for rare childhood tumors in Europe—that is, an annual incidence of less than two cases per million population—melanoma is rare in children (its incidence being around 0.7–0.8 per million a year in the first decade of life), but not in adolescents, that is, patients between 15 and 19 years (with more than 10 per million in the second decade) . Melanoma may actually qualify as an “orphan” disease in the world of pediatric oncology: published series have mainly been retrospective single‐institution series and the few prospective studies conducted to date included a very small number of cases; scanty biological information is available on pediatric melanoma; there are no clinical protocols dedicated to pediatric melanoma patients, so their treatment is generally extrapolated from the approach applied to adults.…”

Characteristics of Spitz nevi, Atypical Spitz nevi and Melanomas in patients younger than 20 years

“…Editor The spectrum of Spitzoid tumours and early onset melanomas has been extensively studied during the last few years. 1,2 Although significant progress has been made in the histopathological and clinical description of these lesions, the biologic potential of the most atypical lesions of the spectrum still remains to be elucidated.…”

Cutaneous melanoma in adolescents and young adults

“…There is still a lack of awareness that young people can develop melanoma, and this frequently leads to diagnostic delays and to cases being referred inappropriately to experienced oncology centers . Published series are mainly retrospective single‐institution series . Very few prospective studies have been conducted to date, and only on small samples of patients .…”

“…25 Published series are mainly retrospective single-institution series. 15,[25][26][27][28][29][30][31][32][33][34][35][36][37][38] Very few prospective studies have been conducted to date, and only on small samples of patients. [39][40][41] The EXPeRT recently published an interesting observational analysis on a sample that included a relatively large number of melanoma cases (219 patients aged 0-18 years) prospectively registered and treated within a short period of time (2002)(2003)(2004)(2005)(2006)(2007)(2008)(2009)(2010)(2011)(2012) in different countries (Italy, Poland, France, Germany, and Israel).…”