A Rare Soft-Tissue Tumor in a 15-Year-Old Boy With Tuberous Sclerosis Complex

Lindsey Oudijk, Elodie J. Mendels, Jeffrey Damman
2019 American journal of dermatopathology  
A 15-year-old boy known with tuberous sclerosis complex with a TSC2 mutation and clinical features of hypopigmented macules, facial angiofibromas, mental retardation, epilepsy, angiomyolipoma, and cardial rhabdomyoma presented at our pediatric dermatology clinics. He had a gradually increasing large soft multinodular tumor in the neck ( Fig. 1) , which had been present for 5 months. At times, the lesion was painful, and the patient noticed purulent discharge. The tumor measured 5.3 · 3.5 cm and
more » ... showed an irregular surface with comedo-like/cystic openings. The patient requested removal of the tumor for cosmetic reasons. On gross pathology, the specimen showed a diffuse, pale fibrotic appearance, with fibrosis extending into the subcutaneous fat ( Fig. 2A) . Histopathologic examination of the lesion revealed 3 components: abundant thick collagen bundles in the dermis and underlying adipose tissue; concentric perifollicular, perieccrine, and perivascular fibrosis; and variable-sized epithelial cysts and dilated openings (Figs. 2B-D).
doi:10.1097/dad.0000000000001388 pmid:32437113 fatcat:uujiwjh6jjh2bcjekqtqgesz7q