TY - JOUR
T1 - Cutaneous malignant melanoma in children and adolescents treated in pediatric oncology units
AU - Réguerre, Yves
AU - Vittaz, Marie
AU - Orbach, Daniel
AU - Robert, Caroline
AU - Bodemer, Christine
AU - Mateus, Christina
AU - Plantaz, Dominique
AU - Plouvier, Emmanuel
AU - Lutz, Patrick
AU - Rakotonjanahary, Josue
AU - Fraitag, Sylvie
AU - Martin, Ludovic
N1 - Publisher Copyright:
© 2016 Wiley Periodicals, Inc.
PY - 2016/11/1
Y1 - 2016/11/1
N2 - 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.
AB - 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.
KW - adolescent
KW - child
KW - malignant skin melanoma
KW - spitzoid melanoma
UR - http://www.scopus.com/inward/record.url?scp=84988317003&partnerID=8YFLogxK
U2 - 10.1002/pbc.26113
DO - 10.1002/pbc.26113
M3 - Article
C2 - 27348579
AN - SCOPUS:84988317003
SN - 1545-5009
VL - 63
SP - 1922
EP - 1927
JO - Pediatric Blood and Cancer
JF - Pediatric Blood and Cancer
IS - 11
ER -