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Oncology Letters
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Print ISSN: 1792-1074 Online ISSN: 1792-1082
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September 2012 Volume 4 Issue 3

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International Journal of Molecular Medicine is an international journal devoted to molecular mechanisms of human disease.

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Case Report

Fibrosarcomatous pigmented dermatofibrosarcoma protuberans: A case report with review of the literature

  • Authors:
    • Mitsuaki Ishida
    • Hidetoshi Okabe
  • View Affiliations / Copyright

    Affiliations: Department of Clinical Laboratory Medicine and Division of Diagnostic Pathology, Shiga University of Medical Science, Otsu, Shiga 520-2192, Japan
  • Pages: 390-392
    |
    Published online on: June 19, 2012
       https://doi.org/10.3892/ol.2012.765
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Abstract

Dermatofibrosarcoma protuberans (DFSP) is a relatively rare low-grade sarcoma that occasionally exhibits melanin-containing spindle cells within the tumor. Fibrosarcomatous DFSP (FS-DFSP) is a variant that is characterized by areas which are histopathologically indistinguishable from fibrosarcoma. In the present study, we describe a case of fibrosarcomatous pigmented DFSP and review the clinicopathological features of this extremely rare lesion. A 51-year-old male presented with a slow-growing nodular cutaneous mass in his left upper arm. Histopathologically, the resected tumor was comprised of pigmented DFSP in approximately 20% of the tumor, with the remaining area further covered by a fibrosarcomatous component. A review of the clinicopathological features of the five previously reported cases as well as the present case indicated that this lesion mainly affects middle-aged males and occurs mostly in the extremities. Melanin-containing spindle cells are present only in the conventional DFSP component. The prognosis appears to be poor; in the six cases reviewed, four demonstrated multiple metastases and three succumbed to the disease. Our analyses revealed that the presence of a fibrosarcomatous component in pigmented DFSP is associated with aggressive behavior; therefore, careful assessment for the presence of a fibrosarcomatous component is necessary in the diagnosis of this disease.
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1 

Weyers W, Mentzel T, Kasper RC, et al: Dermatofibrosarcoma protuberans. World Health Organization Classification of Tumours. Pathology and Genetics of Skin Tumours. LeBoit PE, Burg G, Weedon D and Sarasain A: IARC Press; Lyon: pp. 259–261. 2006

2 

Dupress WB, Langloss JM and Weiss SW: Pigmented dermatofibrosarcoma protuberans (Bednar tumor): A pathologic, ultrastructural, and immunohistochemical study. Am J Surg Pathol. 9:630–639. 1985. View Article : Google Scholar : PubMed/NCBI

3 

Mentzel T, Beham A, Katenkamp D, Dei Tos AP and Fletcher CDM: Fibrosarcomatous (“high-grade”) dermatofibrosarcoma protuberans: clinicopathologic and immunohistochemical study of a series of 41 cases with emphasis on prognostic significance. Am J Surg Pathol. 22:576–587. 1998.

4 

Onoda N, Tsutsumi Y, Kakudo K, et al: Pigmented dermatofibrosarcoma protuberans (Bednar tumor). An autopsy case with systemic metastasis. Acta Pathol Jpn. 40:935–940. 1990.PubMed/NCBI

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Porter C, Vincetic A, Saleh ME and Goldstein H: Pigmented dermatofibrosarcoma protuberans of the foot with fibrosarcomatous changes: a review and case presentation. J Foot Ankle Surg. 41:186–191. 2002. View Article : Google Scholar : PubMed/NCBI

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Suehara Y, Yazawa Y and Hitachi K: Metastatic Bednar tumor (pigmented dermatofibrosarcoma protuberans) with fibrosarcomatous change: a case report. J Orthop Sci. 9:662–665. 2004. View Article : Google Scholar : PubMed/NCBI

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Voth H, Landsberg J, Hinz T, et al: Management of dermatofibrosarcoma protuberans with fibrosarcomatous transformation: an evidence-based review of the literature. J Eur Acad Dermatol. 25:1385–1391. 2011. View Article : Google Scholar : PubMed/NCBI

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Copy and paste a formatted citation
Spandidos Publications style
Ishida M and Okabe H: Fibrosarcomatous pigmented dermatofibrosarcoma protuberans: A case report with review of the literature. Oncol Lett 4: 390-392, 2012.
APA
Ishida, M., & Okabe, H. (2012). Fibrosarcomatous pigmented dermatofibrosarcoma protuberans: A case report with review of the literature. Oncology Letters, 4, 390-392. https://doi.org/10.3892/ol.2012.765
MLA
Ishida, M., Okabe, H."Fibrosarcomatous pigmented dermatofibrosarcoma protuberans: A case report with review of the literature". Oncology Letters 4.3 (2012): 390-392.
Chicago
Ishida, M., Okabe, H."Fibrosarcomatous pigmented dermatofibrosarcoma protuberans: A case report with review of the literature". Oncology Letters 4, no. 3 (2012): 390-392. https://doi.org/10.3892/ol.2012.765
Copy and paste a formatted citation
x
Spandidos Publications style
Ishida M and Okabe H: Fibrosarcomatous pigmented dermatofibrosarcoma protuberans: A case report with review of the literature. Oncol Lett 4: 390-392, 2012.
APA
Ishida, M., & Okabe, H. (2012). Fibrosarcomatous pigmented dermatofibrosarcoma protuberans: A case report with review of the literature. Oncology Letters, 4, 390-392. https://doi.org/10.3892/ol.2012.765
MLA
Ishida, M., Okabe, H."Fibrosarcomatous pigmented dermatofibrosarcoma protuberans: A case report with review of the literature". Oncology Letters 4.3 (2012): 390-392.
Chicago
Ishida, M., Okabe, H."Fibrosarcomatous pigmented dermatofibrosarcoma protuberans: A case report with review of the literature". Oncology Letters 4, no. 3 (2012): 390-392. https://doi.org/10.3892/ol.2012.765
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