Cutaneous melanoma in children and adolescents: The EXPeRT/PARTNER diagnostic and therapeutic recommendations

Abstract

Cutaneous melanoma is rare in children and, like other very rare pediatric tumors, it suffers from a shortage of knowledge and clinical expertise. The clinical management of pediatric melanoma is often challenging. Its clinical and pathological diagnosis may be difficult, and there is no standard treatment. In the absence of specific treatment guidelines, young patients are generally treated following the same principle as for adults, but concern remains about their access to clinical trials and new drugs, which have been shown to dramatically change the natural history of advanced melanoma. This paper presents the internationally recognized recommendations for the diagnosis and treatment of children and adolescents with cutaneous melanoma, established by the European Cooperative Study Group for Pediatric Rare Tumors

Rare Tumors in Children and Adolescents – the STEP Working Groupʼs Evolution to a Prospective Registry

“…Similar cooperative analyses have also been established for colorectal carcinoma and malignant melanoma [16,23]. These works have been the basis for establishing closer international collaboration with the funding of the European Commission as part of the ExPO-r-Net (European Expert Pediatric Oncology Reference Network for Diagnostics and Treatment) and partner projects [1,3,5,16,17,26,34,37]. By joining international groups into one registry, it becomes possible to gain further knowledge, especially for very rare tumors.…”

Examining the role of wide excision margins in pediatric melanoma: A National Cancer Database analysis

“…Prior literature suggests that pediatric melanoma behaves distinctly from melanoma in adults, suggesting that a one‐size (or margin width) fits‐all approach may not be appropriate for all age groups 13,18,19 . Although it is difficult to perform randomized trials in the pediatric population, given the rarity of the disease as highlighted in the recent EXPeRT/PARTNER recommendations for cutaneous melanoma in children and adolescents, retrospective and database studies may shed light on the impact of excision margin width, specifically in the pediatric melanoma population 5,7,8 . Using a large national cohort of children with melanoma, we found that primary melanoma tumor resection with narrow margins (≤1 cm) in pediatric melanoma was associated with similar OS compared to wider (>1 cm) margins.…”

“…Surprisingly, our additional finding that there was no interaction between excision margin width and Breslow depth further suggests that the effect of excision margin width on OS does not differ based on Breslow depth. An adequately powered prospective trial to determine optimal surgical margins for children would be impractical due to the rarity of the disease, and some may argue lack equipoise given the data currently available in the adult population 5 . Instead, retrospective studies such as ours may help provide valuable information to guide clinicians who care for children with melanoma.…”

“…Current adult guidelines recommend standard excision margin widths based on Breslow depth, though a large, ongoing international study is currently examining the role for 1 cm margins for adult melanoma patients regardless of Breslow depth and may further define the safety of smaller margins for melanoma patients (NCT02385214, NCT03860883) 30 . In contrast to the data available in adult patients, the rarity of pediatric melanoma makes it difficult to conduct trials among this population, leading to a paucity of data on excision margins specific to pediatric melanoma 5 . Given their smaller body surface area, children may benefit from minimizing surgical margins to improve wound closure and postoperative healing.…”

“…[4][5][6][7][8][9] Pediatric melanoma may not present with typical clinical features seen in adults, particularly when present in pre-pubertal children, and may present in the setting of other conditions such as xeroderma pigmentosum or congenital melanocytic nevi. 5,[10][11][12] Additionally, histologic subtypes more prevalent in children such as spitzoid melanoma are often difficult to diagnose and distinguish from benign lesions. 4,13 Children are also more likely to present with thicker lesions, nodal involvement, and distant metastases, though survival is similar to adults.…”