Abstract

Abstract A 14-year-old boy was referred to dermatology with a new bleeding lesion on his right neck that had grown rapidly. There was no preceding history of trauma. Physical examination revealed a discrete eroded pink nodule measuring 10 × 9 mm on his right neck. Dermoscopic examination showed a central area of ulceration, with polymorphous vessels, white lines and multiple shades of pink and milky-white areas. This was excised. Histology confirmed the diagnosis of ulcerated superficial spreading malignant melanoma with a Breslow thickness of 4.8 mm (pT4b). BRAF v600e mutation was present. Initial staging with sentinel lymph node biopsy and whole-body positron emission tomography/­computed tomography were clear, but 6 months after presentation, he developed right-sided cervical lymphadenopathy and a parotid mass that was confirmed to be metastatic disease. Following a right parotidectomy and neck dissection, he was commenced on adjuvant treatment with dabrafenib and trametinib. He remains under close follow-up. Less than 1% of all melanomas are diagnosed in patients younger than 20 years of age. Most paediatric cases occur in adolescence (between 15 and 19 years of age). The majority arise sporadically. Risk factors for melanoma in childhood include giant congenital melanocytic naevi, personal history of malignancy, family history of melanoma, multiple (> 50) melanocytic naevi, history of sunburn or tanning-bed use, immunosuppression or genodermatoses (e.g. xeroderma pigmentosum). Most clinicians are familiar with the ‘ABCDE’ tool (asymmetry, border irregularity, colour variegation, diameter > 6 mm, evolution) used to help with the recognition of melanomas; however, melanoma in children often presents in an atypical fashion. Paediatric melanomas often lack pigment (amelanotic) and are nodular, and so may be misdiagnosed. Because of this, diagnosis may be delayed and patients often present with thicker tumours and lymph node involvement. To facilitate earlier diagnosis, modified ABCD detection criteria have been proposed for paediatric melanoma (amelanosis, bleeding/bump, colour uniformity, de novo development) that can be used in conjunction with the established ABCDE approach. The CUP criteria have also been described (colour that is pink/red/changing, ulceration/upward thickness, pyogenic granuloma-like lesions/pop-up of new lesions). Evolution remains an important point to consider when suspecting melanoma in patients of any age. Management and staging of paediatric melanoma is adopted from the American Joint Committee on Cancer melanoma staging system guidelines for adult melanoma. Currently, there are no specific guidelines for the management of melanoma in children. The mainstay of treatment is surgical resection. Sentinel lymph node biopsy should be considered with ulceration or a Breslow thickness of > 0.75 mm. Patients with stage III and IV melanoma should be considered for adjuvant immunotherapy. All patients with melanoma should be followed-up with regular skin examinations.

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