Malignant transformation of multiple adnexal tumors in a Filipino-American patient with Brooke-Spiegler Syndrome: A case report

ABSTRACT

@#<p style="text-align: justify;"><strong>INTRODUCTION:</strong> Trichoepithelioma is a benign hamartoma which may exhibit similar clinical and histopathological features with basal cell carcinoma. Since prognosis is dissimilar, differentiating between them is important. Literature reveals fifteen reports of malignant transformation of multiple trichoepitheliomas into basal cell carcinoma, with none in the local setting. In Brooke-Spiegler syndrome, the incidence rate of malignant transformation of benign neoplasms has been reported in 5-10 % of patients.</p><p style="text-align: justify;"><strong>CASE REPORT:</strong> A 53-year-old Filipino-American female presented with multiple discrete to coalesced, well-defined, skin-colored to hyperpigmented, smooth, dome-shaped, rubbery papules and nodules on the face since childhood. Throughout the years, lesions increased in number and size and spread to the scalp, chest, abdomen, back, upper and posterior right thigh. Some developed ulceration, telangiectasia and pigmentation.</p><p style="text-align: justify;">Chest radiography, radiographs of the jaw, whole abdominal ultrasound, cranial CT scan, ophthalmology and otorhinolaryngology assessments were normal. Based on clinical and histopathologic findings, the final diagnosis was Brooke-Spiegler syndrome with transformation into basal cell carcinoma. Carbon dioxide (CO2) laser was used to excise large and ulcerated lesions with good cosmetic results.</p><p style="text-align: justify;"><strong>CONCLUSION:</strong> A case of a Filipino-American adult female diagnosed clinically and histologically with Brooke-Spiegler syndrome with transformation into basal cell carcinoma was presented. Given the similarities in clinical and histopathologic features of trichoepithelioma and basal cell carcinoma, accurate diagnosis should be made because of their difference in prognosis. The use of an acceptable treatment modality such as carbon dioxide (CO2) laser in this case is an important emerging fi eld of study.</p><p style="text-align: justify;"><strong>KEYWORDS:</strong> trichoepithelioma, basal cell carcinoma, Brooke-Spiegler syndrome, malignant transformation, carbon dioxide laser</p>