Despite recent advances in treatment and surveillance, metastatic melanoma still carries a poor prognosis. Large/giant congenital melanocytic nevi (CMNs) constitute a known risk factor for the condition, with the greatest risk for malignant transformation thought to be during childhood (median age at diagnosis of 3 years in a previous cohort). Herein, we present the case of a 30-year-old male who, after undergoing multiple excision/grafting procedures for a giant CMN as a child, was diagnosed with an NRAS-mutant, MDM2-amplified metastatic melanoma more than 20 years later. Response to ipilimumab/nivolumab immunotherapy, cisplatin/vinblastine/temozolomide chemotherapy, and nivolumab/relatlimab immunotherapy was poor. This case highlights the importance of lifetime monitoring with once-yearly dermatological examination (including lymph node palpation) in large/giant CMN patients, as well as the need for further clinical trials evaluating novel therapies for NRAS-mutant melanoma.

This study assessed self-reported health-related quality of life and psychological adjustment in 43 adolescents and young adults (ages in years: 14-24, M = 17.6, SD = 2.2) with congenital melanocytic nevi (CMN) and examined associations with sociodemographic variables, characteristics of the CMN, perceived social reactions, and cognitive emotion regulation strategies. Outcome measures included the Pediatric Quality of Life Inventory™ 4.0 and the Strengths and Difficulties Questionnaire. Findings suggest impaired psychosocial health and psychological adjustment in youth with CMN compared to community norms. Impairments were associated with higher age of participants, lower socioeconomic status, visibility of the skin lesion, perceived stigmatization, poorer perceived social support, and maladaptive cognitive emotion regulation strategies (self-blame, rumination, and catastrophizing), but not with sex of participants, extent of the skin lesion, and surgical removal of the nevus. Implications for clinical practice and future research are discussed.

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