BRAF-Mutated and Morphologically Spitzoid Tumor Arising Within a Congenital Melanocytic Nevus in a Pediatric Patient:...

B-Raf proto-oncogene, serine/threonine kinase (BRAF)-mutated and morphologically spitzoid tumors (BAMS) are a recently described subset of melanocytic neoplasms characterized by spitzoid histomorphology in the presence of canonical BRAF mutations, most commonly BRAF V600E (valine-to-glutamic acid substitution at codon 600). These lesions may clinically and histologically mimic Spitz nevus, atypical Spitz tumor, or spitzoid melanoma, creating diagnostic challenges. We report the case of an 11-year-old previously healthy female with a congenital melanocytic nevus who developed a new papular lesion within the nevus. Histopathologic evaluation revealed a spitzoid melanocytic proliferation with atypical features. Immunohistochemistry supported melanocytic differentiation with a low proliferative index, and molecular testing detected a BRAF V600E mutation. An integrated clinicopathologic and molecular assessment established the diagnosis of BAMS. The patient underwent complete surgical excision with planned close follow-up. This case highlights the importance of molecular testing in spitzoid melanocytic lesions and emphasizes the need for accurate classification to guide appropriate management.