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  • Melanoma in the pediatric population can present distinctly from adult disease, such as Spitz melanoma or melanoma associated with congenital melanocytic nevi.

  • Pediatric patients with basal cell carcinoma or squamous cell carcinoma should be thoroughly evaluated for predisposing risk factors.

  • Patients with genodermatoses, including those with photosensitivity-associated DNA repair deficits, melanin synthesis defects, and tumor predisposition syndromes, should be closely monitored because of the increased risk of developing skin cancer.


  • Unlike in adult disease, melanoma in pediatric patients can present as an amelanotic macule, papule, or nodule that may be flesh-colored, pink, or red.


  • Typical features of adult melanoma are not always present in the pediatric population. Incorporate pediatric-specific criteria when assessing lesions suspicious for melanoma in pediatric patients.

  • Pediatric melanoma often presents with a higher histopathologic stage and a higher Breslow depth compared with adult melanoma, perhaps associated with delays in diagnosis.


  • Although pigmented lesion onset or change in adults is considered to be concerning for melanoma, these changes may represent the natural history of melanocytic nevi in children.

  • Photoprotection is vital to mitigate skin cancer risk in pediatric patients with predisposing risk factors or genodermatoses.


Skin cancer in the pediatric population is rare and presents with important differences from that seen in adult disease. Children and adolescents with cutaneous malignancies have risk factors, clinical presentations, and therapeutic tolerability and responses particular to their demographic. This chapter provides a detailed review of melanoma, basal cell cancer, and squamous cell cancer in the pediatric population, including risk factors and genetic conditions associated with the development of these rare diseases. The chapter also describes genodermatoses in which patients are at increased risk of developing cutaneous malignancies. Lastly, the chapter includes a discussion of primary cutaneous lymphomas and soft tissue tumors in children and adolescents.


Pediatric melanoma is a rare entity, with only 1.3% of cutaneous melanoma cases affecting children under 18 years of age.1 Melanoma in the pediatric population tends to present in adolescence, with 79% of pediatric cases found to develop between the ages of 15 and 19.2 Although the incidence of pediatric melanoma had previously been on the rise after 1970, in more recent years, the incidence of melanoma in children the United States has declined, decreasing about 11% per year since 2004 through 2010.3-5 There are three main types of pediatric melanoma: conventional melanoma, Spitz melanoma, and melanoma arising from congenital melanocytic nevus (CMN), in addition to other more esoteric presentations with distinct etiology and pathology, including acral, ocular, and congenital melanoma.6

Risk Factors

Although melanoma is rare in the pediatric population, genetic, environmental, and immunologic risk factors should be considered in these patients. It is important to note that although certain ...

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