Giant congenital melanocytic nevus treated with trametinib

Adnan Mir, Nnenna G. Agim, Alex A. Kane, Shellie C Josephs, Jason Y. Park, Kathleen Ludwig

Research output: Contribution to journalArticlepeer-review

24 Scopus citations


Giant congenital nevi are melanocytic proliferations of the skin that may be complicated by melanoma, neurocutaneous melanocytosis, pain, pruritus, and disfigurement. Current treatment options include surgical resection and medical management of associated symptoms. There is limited efficacy in these modalities. No effective pharmacologic treatments are available for the treatment of these lesions. We present the case of a 7-year-old girl with a giant congenital melanocytic nevus that had an AKAP9-BRAF fusion and was treated with trametinib, which resulted in rapid resolution of the patient's lifelong, intractable pain and pruritus as well as dramatic improvement in the extent of her nevus.

Original languageEnglish (US)
Article numbere20182469
Issue number3
StatePublished - Mar 2019

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health


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