Squamous Cell Carcinoma Arising in a Nevus Sebaceous of Jadassohn in a 9-Year-Old Girl: Treatment Using Mohs Micrographic Surgery with Literature Review

Abstract

The authors have indicated no significant interest with commercial supporters. J adassohn first described nevus sebaceous of Jadassohn (NSJ), a localized skin malformation, in 1895. 1 Mehregan and Pinkus 2 outlined three clinical stages of NSJ development. During the first stage, in infancy and childhood, the NSJ usually appears as a smooth, alopecic, orange-yellowish skin thickening. A second stage occurs in puberty, when the NSJ thickens and contains verrucous papules, plaques, or both. A third stage may occur in adulthood characterized by development of benign and malignant tumors in the original nevus.In addition to basal cell carcinomas (BCCs), trichoblastomas, squamous cell carcinomas (SCCs), and basosquamous carcinomas, other tumors described in association with NSJ are syringocystadenoma papilliferum, apocrine cystadenoma, apocrine carcinoma, adnexal carcinomas, tubuloglandular sweat gland carcinoma, 3 leiomyoma, 4 metastasizing adenocarcinoma, 5 and sebaceous carcinoma. 6 SCC rarely occurs in NSJ and even more rarely during childhood. We report a SCC occurring within a NSJ in a child. Case ReportA 9-year-old fair-skinned Caucasian girl presented with a 5-Â 7-mm erythematous ulcerated nodule that had rapidly grown over 2 to 3 weeks on the left temple within a congenital cluster of yellowish papules ( Figure 1A). The patient was otherwise healthy, with no skeletal, ocular, or neurological abnormalities. There was no lymphadenopathy in the preauricular area or neck. There was no preceding history of a wart, skin cancer, or photosensitivity. The remainder of the personal and family history was not suggestive of any cancer-prone genodermatoses. Differential diagnoses for the nodule included Spitz nevus, wart, or a secondary tumor occurring in a congenital NSJ.Histopathology of a skin biopsy showed markedly atypical spindle squamous cells originating from the epidermis and replacing most of the dermis ( Figure 2A). Clusters and nests of markedly atypical epidermal cells were seen to infiltrate a desmoplastic stroma. Foci of keratinization and dyskeratosis were noted. At its deepest area of invasion, the tumor contained a prominent spindle cell component ( Figure 2B). Tumor cells had pleomorphic, hyperchromatic nuclei with occasional prominent nucleoli and occasional mitotic figures ( Figure 2C). These findings were consistent with a moderately to poorly differentiated infiltrating SCC. Immunohistochemical stains revealed strong positive staining of tumor cells with antibodies to cytokeratin 5/6 and pancytokeratin. Staining was negative with antibodies to S-100,

Dermoscopic Analysis of Nevus Sebaceus of Jadassohn: A Study of 13 Cases

“…Benign tumors [12,27,28,29,30,31,32] are the most common neoplasms, including syringocystadenoma papilliferum, apocrine cystadenoma, trichoblastoma, trichilemmal cysts, and keratoacanthoma. Although less common, malignant degeneration [33,34,35,36,37,38,39,40,41,42,43,44,45,46,47] occurs, with a lifetime risk of 5-20%. The majority of these tumors are basal cell carcinomas, but squamous cell, sebaceous, and apocrine carcinomas have also been recognized [2,13].…”

Giant Nevus Sebaceus

“…Basal cell carcinoma has been documented in 14 prepubescent children as young 5 years of age (mean, 10.5 years; range, 5 to 17 years) and squamous cell carcinoma in a 9-year-old. 16,18,24 Although rare, the risk of early malignant change should be discussed with the family when considering the timing of surgical treatment. An additional consideration is the need for tissue expansion and multiple surgical procedures, as delaying multiple surgeries until the child is older has been shown to negatively affect self-esteem.…”

Eccrine Poroma Arising within Nevus Sebaceous

“…Full-thickness excision should be performed [ 5 ]. Mohs micrographic surgery may be offered in cases of nevus sebaceous with concomitant malignant tumor [ 12 ]. Timing for excision is controversy [ 5 ].…”

“…Timing for excision is controversy [ 5 ]. Due to the low incidence of malignant tumor arising on nevus sebaceous, prophylactic excision is unnecessary [ 12 ]. Several dermatologists suggest that it is best to perform excision when there is evidence of secondary tumor or when nevus sebaceous has a great impact on cosmetic appearance [ 12 , 13 , 14 ].…”

“…Due to the low incidence of malignant tumor arising on nevus sebaceous, prophylactic excision is unnecessary [ 12 ]. Several dermatologists suggest that it is best to perform excision when there is evidence of secondary tumor or when nevus sebaceous has a great impact on cosmetic appearance [ 12 , 13 , 14 ]. Alternative treatments for nevus sebaceous include fractional laser, dermabrasion, or photodynamic therapy that have variable outcomes, as nevus sebaceous may not be completely removed through these modalities [ 5 , 15 , 16 , 17 , 18 , 19 , 20 ].…”