Acta Dermato-Venereologica 99-12CompleteContent

1182 SHORT COMMUNICATION Microcystic Adnexal Carcinoma Originating in a Nevus Sebaceous: A Case Report of a 16-year-old Boy Rasha HASHEM 1,2 , Nahreen TYNNGÅRD 3,4 , Katarzyna LUNDMARK 2,5 and Lars FALK 1–3 Department of Dermatology and Venereology, Linköping University Hospital, 2 Department of Clinical and Experimental Medicine, Linköping University, Linköping, 3 Research and Development Unit in Region Östergötland, 4 Department of Medical and Health Sciences, Linköping University, and 5 Department of Clinical Pathology, Linköping University Hospital, Linköping, Sweden. E-mail: rasha.hashem@ regionostergotland.se 1 Accepted Aug 6, 2019; E-published Aug 6, 2019 Nevus sebaceous (NS) is a congenital hamartoma, com- prised of abnormal epidermal and dermal components including both the adnexal (follicular pilosebaceous and glandular) and epithelial parts in different proportions (1). NS affects mainly the scalp and the estimated pre- valence in newborn infants is 0.3% (2, 3). Normally the hamartoma remains benign. However, NS transforms into different types of neoplastic tumors in about 10–20% of cases (4). The majority of these secondary neoplasms is benign and occurs in adults older than 40 years of age (3). The rate of malignant transformation of NS has been reported to be up to 3% (3, 5) and the most common tumor is basal cell carcinoma (BCC) (3, 5). Malignant transformation in children and adolescents is rare. In an extensive literature review by Moody et al. (4), they reviewed 4,923 cases of NS and found that only 24 of 204 cases of malignancies such as BCC, squamous cell carcinoma and keratoacanthoma had occurred in indi- viduals younger than 18 years old. Microcystic adnexal carcinoma (MAC) is a rare cutaneous neoplasm of the skin adnexa, which very rarely originates within a NS, although it has been reported in adults (5–8). To the best of our knowledge MAC originating in a NS in a child or an adolescent has not previously been reported. CASE REPORT A 16-year-old boy, born with a yellow-grey NS on the parietal area of the scalp, attended the primary health care center in May 2016 due to increasing itching in the lesion. Apart from the itching, which commenced 12 months prior to the visit he had no other symptoms, was generally healthy and taking no medications. There was no history of skin cancer in the family. The boy had skin type 1 as graded by Fitzpatrick. He had not been exposed to radiation in the head and neck region. Clinical and dermatoscopic examination revealed a 20 × 35 mm NS of classical appearance and the lesion was thus deemed to be benign. However, the patient noticed that the lesion was growing slightly and that the itching intensified. He therefore re-attended 6 months later and was examined again by a general practitioner who on ocular inspection did not find any abnormal changes and decided that excision of the lesion was not necessary. Because of the symptoms, however, the patient was referred to the Department of Dermatology at the Linköping University Hospital for a second opinion. The dermatologist found no abnormal clinical or dermatoscopic changes within the lesion (Fig. 1). Due to continued itching, however, the lesion was excised two months later. The histopathological examination showed a NS with an area containing keratinous cysts, solid nests and strands of basaloid cells and duct-like structures in a sclerotic stroma, consistent with MAC. Focal perineural invasion was present. doi: 10.2340/00015555-3272 Acta Derm Venereol 2019; 99: 1182–1183 Fig. 1. The lesion located on the scalp 2 months before excision. Immunohistochemistry showed low proliferative activity (Ki67, Dako Denmark, dilution 1:200) and sparse nuclear p53 expression with low to moderate intensity (Dako, dilution 1:200), consistent with MAC (9). The lesion was very close to the side margin (Fig. 2). The initial surgical excision was done with 2 mm margins, and hence re-excision was needed. The wide local excision (WLE) was done with approximately 2 cm margin to ensure cancer clearance. No residual NS or MAC was present in the WLE specimen. Cervi- cal lymph nodes were not palpable. The patient is doing well, with no signs of recurrence 2 years after surgery. Magnetic resonance imaging showed no remaining lesion. DISCUSSION MAC is a rare locally aggressive cancer of the skin ad- nexa. It was first described by Goldstein et al. in 1982 to originate from a pluripotent adnexal keratinocyte (10). According to a review article by Gordon et al. (11) and a study by Yu et al. (12) MAC occurs most often in middle-aged to older Caucasian individuals and has an incidence of between 1.6 and 6.5 per 10,000,000 indivi- duals (12). Only 4 cases of MAC arising from NS have been reported and all were identified in adults aged 75, 26, 62 and 65 years, respectively (5–8). To our knowledge this is the first reported case of MAC originating in a NS in an adolescent. This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation © 2019 Acta Dermato-Venereologica.

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