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Pathological Case of the Month FREE

Teri A. Kahn, MD; Maritza O. Liranzo, MD; Allison T. Vidimos, MD; Frank A. Papay, MD; Wilma F. Bergfeld, MD
Arch Pediatr Adolesc Med. 1996;150(5):549-550. doi:10.1001/archpedi.1996.02170300103021.
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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

REFERENCES

Annessi G, Cimitan A, Girolomoni G, Giannetti A.  Congenital dermatofibrosarcoma protuberans . Pediatr Dermatol . 1993;;10:40-42.
McKee PH, Fletcher CD.  Dermatofibrosarcoma protuberans presenting in infancy and childhood . J Cutan Pathol . 1991;;18:241-246.
Taylor HB, Helwig EB.  Dermatofibrosarcoma protuberans: a study of 115 cases . Cancer . 1962;;15:717-725.
Kamino H, Jacobson M.  Dermatofibroma extending into the subcutaneous tissue: differential diagnosis from dermatofibrosarcoma protuberans . Am J Surg Pathol . 1990;;14:1156-1164.
Connelly JH, Evans HL.  Dermatofibrosarcoma protuberans: a clinicopathologic review with emphasis on fibrosarcomatous areas . Am J Surg Pathol . 1992;; 16:921-925.
Kutxner H, Germany F.  Expression of the human progenitor cell antigen CD34 (HPCA-1) distinguishes dermatofibrosarcoma protuberans from fibrous histiocytoma in formalin-fixed, paraffin-embedded tissue . J Am Acad Dermatol . 1993;;28:613-617.
Cohen PR, Rappini RP, Farhood Al.  Expression of the human hematopoietic progenitor cell antigen CD34 in vascular and spindle cell tumors . J Cutan Pathol . 1993;;20:15-20.
Hobbs ER, Wheeland RG, Bailin PL, Ratz JL, Yetman RJ, Zins JE.  Treatment of dermatofibrosarcoma protuberans with Mohs micrographic surgery . Ann Surg . 1988;;207:102-107.

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Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal

References

Annessi G, Cimitan A, Girolomoni G, Giannetti A.  Congenital dermatofibrosarcoma protuberans . Pediatr Dermatol . 1993;;10:40-42.
McKee PH, Fletcher CD.  Dermatofibrosarcoma protuberans presenting in infancy and childhood . J Cutan Pathol . 1991;;18:241-246.
Taylor HB, Helwig EB.  Dermatofibrosarcoma protuberans: a study of 115 cases . Cancer . 1962;;15:717-725.
Kamino H, Jacobson M.  Dermatofibroma extending into the subcutaneous tissue: differential diagnosis from dermatofibrosarcoma protuberans . Am J Surg Pathol . 1990;;14:1156-1164.
Connelly JH, Evans HL.  Dermatofibrosarcoma protuberans: a clinicopathologic review with emphasis on fibrosarcomatous areas . Am J Surg Pathol . 1992;; 16:921-925.
Kutxner H, Germany F.  Expression of the human progenitor cell antigen CD34 (HPCA-1) distinguishes dermatofibrosarcoma protuberans from fibrous histiocytoma in formalin-fixed, paraffin-embedded tissue . J Am Acad Dermatol . 1993;;28:613-617.
Cohen PR, Rappini RP, Farhood Al.  Expression of the human hematopoietic progenitor cell antigen CD34 in vascular and spindle cell tumors . J Cutan Pathol . 1993;;20:15-20.
Hobbs ER, Wheeland RG, Bailin PL, Ratz JL, Yetman RJ, Zins JE.  Treatment of dermatofibrosarcoma protuberans with Mohs micrographic surgery . Ann Surg . 1988;;207:102-107.

Correspondence

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