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Encyclopedia of Pathology pp 1–2Cite as

Giant Cell Fibroblastoma

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Part of the book series: Encyclopedia of Pathology ((EP))

Synonyms

Pediatric dermatofibrosarcoma protuberans

Definition

Giant cell fibroblastoma is a rare variant of dermatofibrosarcoma protuberans that affects mainly children in the first decade of life.

Clinical Features

  • Incidence

    Only few cases have been reported in the male external genitalia.

  • Age

    It mainly occurs in children but may also affect adults.

  • Sex

    It involves more frequently male subjects.

  • Site

    Giant cell fibroblastoma may arise in the penis and the scrotum.

  • Treatment

    Treatment is surgical.

  • Outcome

    It is a locally aggressive tumor with tendency to local recurrence. Transformation in fibrosarcoma is exceptional.

Macroscopy

Grossly, giant cell fibroblastoma appears as a firm yellow or gray plaque-like or nodular lesion with rubbery consistency.

Microscopy

Giant cell fibroblastoma involves the dermis and the subcutis, and consists of a proliferation of wavy spindle cells set within a stroma that varies from myxoid to collagenous to sclerotic. The tumor shows infiltrative margins...

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References and Further Reading

  • Eisner, B. H., McAleer, S. J., Gargollo, P. C., Perez-Atayde, A., Diamond, D. A., & Elder, J. S. (2006). Pediatric penile tumors of mesenchymal origin. Urology, 68, 1327–1330.

    Article  Google Scholar 

  • Peard, L., Cost, N. G., & Saltzman, A. F. (2018). Dermatofibrosarcoma protuberans in a male infant. Urology,129, 206–209. pii: S0090–4295(18)31135-X.

    Google Scholar 

  • Woo, L. L., Kuang, W., Zhou, M., & Ross, J. (2006). Giant cell fibroblastoma of the penis. Urology, 68, 427.e5–427.e6.

    Article  Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Translational Research and of New Technologies in Medicine and Surgery, University of Pisa, Pisa, Italy

    Alessandro Franchi

Authors
  1. Alessandro Franchi
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Corresponding author

Correspondence to Alessandro Franchi .

Editor information

Editors and Affiliations

  1. NIJMEGEN, The Netherlands

    J.H.J.M. van Krieken

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Cite this entry

Franchi, A. (2019). Giant Cell Fibroblastoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5001-1

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  • DOI: https://doi.org/10.1007/978-3-319-28845-1_5001-1

  • Received:

  • Accepted:

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-28845-1

  • Online ISBN: 978-3-319-28845-1

  • eBook Packages: Springer Reference MedicineReference Module Medicine

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