Neurofibromatous changes in dermatofibrosarcoma protuberans: a potential pitfall in the diagnosis of a serious cutaneous soft tissue neoplasm

Journal of Cutaneous Pathology
Carrie L KovarikClay J Cockerell

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a low-grade malignant neoplasm that has the potential for aggressive local growth and destruction if not treated appropriately. Although the storiform arrangement of spindle cells in DFSP is relatively characteristic, histologic patterns simulating other benign as well as malignant neoplasms such as dermatofibroma, neurofibroma, malignant fibrous histiocytoma, and atypical fibroxanthoma have been described. We collected and analyzed six cases of probable DFSP in which a specific diagnosis could not be rendered due to the predominant neurofibromatous changes in the histologic sections. In an attempt to reach a definitive diagnosis, the clinical history and physical characteristics of the lesions were taken into account, and all cases were further evaluated using immunostaining for CD34 and S-100 protein. The average age of the patient was 56 years (range 21-80), and the male to female ratio was 1 : 1. The location of lesions included the scalp, neck, back, and abdomen. All cases displayed two distinct histological patterns: (i) a proliferation of spindle cells with wavy nuclei in a loose mucinous stroma suggesting neural differentiation and (ii) a proliferation of spindle cells which int...Continue Reading

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Citations

Feb 9, 2010·The American Journal of Surgical Pathology·Marcia Edelweiss, Anais Malpica
May 11, 2012·Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society·Justin M M Cates, Cheryl M Coffin
Aug 18, 2009·Journal of Neurosurgery·Austin K MattoxD Cory Adamson
Jan 19, 2006·Dermatologic Surgery : Official Publication for American Society for Dermatologic Surgery [et Al.]·Lesley Loss, Nathalie C Zeitouni
Jan 31, 2012·Actas dermo-sifiliográficas·C Serra-GuillénO Sanmartín
Mar 23, 2011·Dermatologic Clinics·Hillary Johnson-Jahangir, Désirée Ratner
Mar 22, 2013·Orbit·Michael P Schittkowski, Arne Wrede
Dec 25, 2012·Journal of the American Academy of Dermatology·David E KurlanderJeremy S Bordeaux
Mar 12, 2016·The Journal of Craniofacial Surgery·Rafael DenadaiKarin Milleni Araujo

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