Abstract

We present a case of a 22-year-old Fitzpatrick Skin Type II male with no personal or family history of skin cancer. He presented to the dermatology clinic for evaluation of a nodule on the chest. He first appreciated the lesion 6 months ago as a raised, skin colored firm nodule which grew rapidly. The physical exam was notable for a 2.5 cm x 2.1 cm shiny red, exophytic, firm tumor of the left chest. An excisional biopsy was performed. Histopathologic sections showed haphazardly arranged dermal fascicles of varying thickness, both interstitial and nodular, which pushed deep into the dermis and the subcutis. While few areas showed relatively bland cells with cigar shaped nuclei without significant atypia, other areas demonstrated marked nuclear pleomorphism and hyperchromasia with atypical mitotic figures. The tumor demonstrated diffuse strong staining with SMA, and focal strong staining with desmin and AE1/3. Notably, S100, SOX10, MelanA, CD34, and ERG were negative. This pattern was found to be consistent with a malignant mesenchymal neoplasm, specifically a leiomyosarcoma. A full body positron emission tomography (PET) scan and a Chest CT did not demonstrate any evidence of lymph node involvement or metastatic disease. The current classification is Stage II (NCCN) and Grade 3 (FNCLCC). The plan for treatment is to proceed with preoperative radiation followed by a wide local excision with surgical oncology for final staging and pediatric oncology follow up. This is an interesting case of a soft tissue sarcoma which is rarely seen in dermatology.

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