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Pediatr Dermatol. 2018 Sep;35(5):e281-e285. doi: 10.1111/pde.13595. Epub 2018 Jul 12.

Aggressive melanoma in an infant with congenital melanocytic nevus syndrome and multiple, NRAS and BRAF mutation-negative nodules.

Author information

1
Department of Dermatology, Oslo University Hospital, Oslo, Norway.
2
Faculty of Medicine, University of Oslo, Oslo, Norway.
3
Department of Plastic and Reconstructive Surgery, Oslo University Hospital, Oslo, Norway.
4
Paediatric Dermatology Department, Great Ormond Street Hospital, London, UK.
5
Department of Neonatal Intensive Care, Oslo University Hospital, Oslo, Norway.
6
Department of Radiology and Nuclear Medicine, Oslo University Hospital, Oslo, Norway.
7
Unit for Pediatric Radiology, Department of Radiology and Nuclear Medicine, Oslo University Hospital, Oslo, Norway.
8
Department of Pathology, Oslo University Hospital, Oslo, Norway.
9
Department of Dermatology, Neurology and Rheumatology, University Hospital of North Norway, Tromsø, Norway.

Abstract

We report the case of a newborn boy with multinodular NRAS and BRAF mutation-negative congenital melanocytic nevi and cerebral lesions compatible with congenital intraparenchymal melanosis. Histopathology from skin lesions showed atypical nodular melanocytic proliferation with marked melanocytic atypia and a large number of mitoses and apoptosis, indicating aggressive proliferation. The child developed several new subcutaneous tumors and multiple internal lesions, which were confirmed to be metastases, and died at 5 months of age. This case may represent an infantile melanoma developing from a giant congenital melanocytic nevus or a congenital melanoma.

KEYWORDS:

neoplasms-malignant; nevi-melanocytic

PMID:
29999207
DOI:
10.1111/pde.13595
[Indexed for MEDLINE]

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