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May 1996

Pathological Case of the Month

Author Affiliations

From the Departments of Dermatology (Drs Kahn, Vidimos, and Bergfeld), Anatomic Pathology (Drs Liranzo and Bergfeld), and Plastic Surgery (Dr Papay), Cleveland Clinic Foundation, Ohio.

Arch Pediatr Adolesc Med. 1996;150(5):549-550. doi:10.1001/archpedi.1996.02170300103021

A HEALTHY 15-YEAR-OLD white female presented with three asymptomatic lesions on her left breast that had been present since birth. The lesions were initially pink maculae that developed into pink nodules after puberty. On physical examination she had two soft, erythematous nodules with atrophic skin and overlying telangiectasia above her left breast. One ill-defined, indurated, violaceous plaque was present above the two soft nodules (Figure 1). There was no hepatosplenomegaly or lymphadenopathy. No other similar cutaneous lesions were identified in this patient. Laboratory investigation revealed a normal complete blood cell count and white blood cell differential. Total cholesterol was mildly elevated at 4.76 mmol/L (184 mg/dL) (normal, 1.94-4.37 mmol/L [75-169 mg/dL]). The biopsy results of one of the nodules are shown in Figure 2.

Diagnosis and Discussion

Congenital Dermatofibrosarcoma Protuberans

Histologic examination revealed a proliferation of atypical plump spindle cells within the dermis extending to the subcutaneous tissue in a