Giant congenital melanocytic nevus with developmental dysplasia of bilateral hip: A rare association

Abstract

Giant congenital melanocytic nevi are rare congenital disfiguring benign neoplasms with a risk of transformation to malignant melanoma. They often present with various extra-cutaneous features. Here, we describe a case of giant melanocytic nevus with developmental dysplasia of bilateral hip, a novel association.

Reconstruction of a Giant Congenital Melanocytic Nevus Defect With a Submental Flap in a Global Health Setting

“…Excision is the first-line treatment option for all large and giant hairy CMN due to the associated risk of malignant transformation, although the magnitude of this risk is debated. GCMN is typically limited to the skin but may invade the underlying structures, including bone and central nervous system [ 7 ]. In this case, the lesion did not invade the underlying structures deeper than the buccal subcutaneous adipose tissue, thus allowing proper excision.…”

Giant congenital melanocytic nevus in a Cameroonian child: a case report

“…CMN are pigmented skin lesions formed by epidermal and dermal-derived nevi cells (also known as melanocytic cells), which can be present at birth or may develop after a few weeks of life [ 1 , 2 , 4 , 7 ]. CMN differs from the acquired form of melanocytic nevus by its presence at birth, tendency to increase in size, and the risk of malignant transformation [ 7 , 8 ]. As previously mentioned, CMN has been classified by many authors based on the size of the lesions.…”

“…The NRAS gene, located on the short arm of chromosome one at position 13.2, provides instructions for making a protein called N-Ras that is involved primarily in regulating cell division [ 11 ]. A mutation of this gene thus leads to a morphological error in the neuro-ectoderm during the fourth and sixth weeks of gestation with uncontrolled growth of melanocytes precursor cells known as melanoblasts, leading to the formation of CMN [ 4 , 7 ]. Furthermore, there is also a molecular component in this pathogenesis, involving the hepatocyte growth factor or scatter factor (HGF/SF), a cytokine partially associated with the control of the development of melanocytes [ 4 , 8 ].…”

“…In fact, it is well established that the disfiguring character of this pathology may have psychosocial implications with deleterious impacts on the self-esteem of affected individuals, especially females [ 4 – 6 ]; this psychological trauma may culminate in social exclusion as was nearly the case in our patient. The most serious organic complications are NCM and the evolution to cutaneous melanoma (1–5% of cases) [ 7 ]. It should be noted that GCMN greater than 40 or 50 cm in diameter and having more than 20 satellite lesions predispose to those severe conditions [ 4 , 9 , 12 ].…”

Giant congenital melanocytic nevus with occipital encephalocele: A very rare association

“…[ 1 ] It is regarded as giant when it involves more than 20 cm in greatest dimension or >9 cm in the scalp or more than 6 cm in the trunk. [ 2 ] In about 82% cases, the disease is axially distributed. Central nervous system (CNS) defects such as spina bifida, meningocele, Dandy Walker malformation may accompany it and thus cause significant morbidity.…”