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Congenital Myxoid and Pigmented Dermatofibrosarcoma Protuberans: A Case Report

Authors

  • May El Hachem M.D.,

    1. Department of Dermatology, Bambino Gesù Children's Hospital Istituto Di Ricovero e Cura a Carattere Scientifico, Rome, Italy
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  • Andrea Diociaiuti M.D., Ph.D.,

    Corresponding author
    • Department of Dermatology, Bambino Gesù Children's Hospital Istituto Di Ricovero e Cura a Carattere Scientifico, Rome, Italy
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  • Ermenegilda Latella M.D., Ph.D,

    1. Department of Dermatology, Bambino Gesù Children's Hospital Istituto Di Ricovero e Cura a Carattere Scientifico, Rome, Italy
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  • Mario Zama M.D,

    1. Department of Maxillofacial and Plastic Surgery, Bambino Gesù Children's Hospital Istituto Di Ricovero e Cura a Carattere Scientifico, Rome, Italy
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  • Caterina Lambiase M.D,

    1. Department of Dermatology, Bambino Gesù Children's Hospital Istituto Di Ricovero e Cura a Carattere Scientifico, Rome, Italy
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  • Loredana Giraldi M.D,

    1. Department of Dermatology, Bambino Gesù Children's Hospital Istituto Di Ricovero e Cura a Carattere Scientifico, Rome, Italy
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  • Tiziana Surrenti M.D,

    1. Department of Dermatology, Bambino Gesù Children's Hospital Istituto Di Ricovero e Cura a Carattere Scientifico, Rome, Italy
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  • Francesco Callea M.D., Ph.D.

    1. Department of Pathology, Bambino Gesù Children's Hospital Istituto Di Ricovero e Cura a Carattere Scientifico, Rome, Italy
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Address correspondence to A. Diociaiuti, M.D., Department of Dermatology, Bambino Gesù Children's Hospital I.R.C.C.S., Piazza Sant'Onofrio, 4-00165 Rome, Italy, or e-mail: andrea.diociaiuti@opbg.net.

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a low-grade, mesenchymal, spindle cell tumor. In addition to the classical form characterized by a storiform pattern of tumor cells, pigmented (Bednar's tumor) and myxoid variants can be observed. Classical DFSP and Bednar's tumor are easily diagnosed. The myxoid variant represents a diagnostic challenge. Pigmented and myxoid variants are rare and thus far have never been reported in association in congenital DFSP. We came across a unique DFSP that was, at the same time, congenital, pigmented, and myxoid. The tumor was surgically excised with broad free margins and no recurrence. The differential diagnosis with other entities such as giant cell fibroblastoma, CD34-positive plaque-like dermal fibroma, superficial plaque-like CD34 DFSP, and neurocristic hamartoma is discussed. The recognition of this hybrid variant of congenital DFSP is important to avoid under- or overtreatment.

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