Giant congenital melanocytic nevus (bathing trunk nevus) associated with lipoma and neurofibroma: Report of two cases

Abstract

Giant congenital melanocytic nevi are rare and occur in about one out of every 2,00,000 to 5,00,000 births. There is a significant association between bathing trunk nevus and neurofibromatosis and lipomatosis. Apart from this, association of bathing trunk nevus with abnormalities like spina bifida occulta, meningocele, club foot and hypertrophy or atrophy of deeper structures of a limb, have been described. We are herewith reporting two cases of bathing trunk nevi. In our first case, an eight-year-old girl presented with a bathing trunk nevus studded with multiple, large nodules. Histopathological examination of the biopsy taken from one nodule revealed features of both neurofibroma and lipoma. To the best of our knowledge, features of both these hamartomas in one nodule of a single patient are probably not reported in the literature. In our second case, a 12-year-old girl presented with bathing trunk nevus and she had spina bifida occulta. She also had lipoma in the lesion of bathing trunk nevus. Both of our patients had satellite melanocytic nevi over the face, forearm, upper back and legs. Our second patient, in addition, had small melanocytic nevi over the medial canthus and sclerocorneal junction of the right eye. By the time this girl presented to us, the melanocytic nevus started fading in color and it had become brownish. We are reporting these cases for their peculiarities and for their rare features.

Alveolar rhabdomyosarcoma arising in a giant congenital melanocytic nevus in an adult – case report with review of literature

“…In the skin, it may develop within a CMN as an extension from the deep soft tissue tumor or by metastatic spread. Sites favored by rhabdomyosarcoma include the orbit, eyelid, head and neck, non‐parameningeal areas, and genitorurinary areas, except the bladder or prostate, while the less common sites include extremities, parameningeal, retroperitoneum, and the trunk 1,2,6 …”

“…Such giant CMNs carry substantial risk of malignant change and are also associated with many serious conditions like neurocutaneous melanosis. Malignant melanoma is the most common malignancy arising within such nevi, and rhabdomyosarcoma is one of the rare but usually fatal malignancies arising within giant CMNs 2,3 …”

“…Malignant melanoma is the most common malignancy arising within such nevi, and rhabdomyosarcoma is one of the rare but usually fatal malignancies arising within giant CMNs. 2,3 We report a case of alveolar rhabdomyosarcoma arising in a giant CMN in an adult with fatal outcome, and review the literature.…”

Insight into evolution of a giant congenital nevomelanocytic nevus over 14 years

“…[5] These nevi might be associated with several other conditions namely neurocutaneous melanosis, rhabdomyosarcoma, liposarcoma, tethered cord syndrome, diffuse lipomatosis, structural brain malformations, hypertrophy of skull bones, limb atrophy, musculoskeletal asymmetry, von Recklinghausen disease and vitiligo. [6,7] Spontaneous regression occurs rarely in pigmented lesions and this process may be associated with the development of a depigmented halo or vitiligo. [8] Discrete dermal nodular proliferations commonly referred to as "proliferative nodules," "atypical dermal nodules," "atypical epithelioid tumors in congenital nevi" or "proliferative dermal lesions" can be identified in congenital nevi.…”

Giant congenital nevus associated with papillary thyroid cancer: the first case report

“…Several developmental abnormalities have been reported as associated with GCN. These include leptomeningeal melanocytosis, 7 spina bifida, 8 Chiari II malformation, 9 lipoma and neurofibroma, 10 Proteus syndrome, 11 brain structural malformations, multiple lipomatosis, and Dandy–Walker syndrome 12…”