Pediatric pigmented dermatofibrosarcoma protuberans (Bednar tumor): Case report and review of the literature with emphasis on the differential diagnosis

• Type: Article in International Scientific Journal
• Date: 2002

Authors

Reis, JS (Author) Other; Milanezi, F (Author) Other; Ferro, J (Author) Other; Fernando Schmitt (Author) FMUP

Journal

Title: Pathology Research and Practice

Vol. 198

Pages: 621-626

ISSN: 0344-0338

Publisher: Elsevier

Indexing

ISI Web of Knowledge - 15 Citations

Scopus - 27 Citations

Other information

Authenticus ID: P-000-RQY

DOI: 10.1078/0344-0338-00311

Abstract (EN): Dermatofibrosarcoma protuberans (DFSP) is a fibrous tumor of intermediate malignant potential that usually affects the trunk of young to middle-aged adults. On histological examination, it is characterized by a monomorphous population of spindle cells arranged in a storiform or cartwheel pattern. Bednar tumor (BT), formerly known as storiform pigmented neurofibroma, is currently considered the pigmented variant of DFSP due to the histological and cytogenetic similarities between these two lesions. There are very few reports on BT affecting pediatric patients. We describe a case of BT affecting the dorsal aspect of the left forearm of a 6-year-old-male patient and emphasize the diagnostic clues to distinguish this unusual cutaneous neoplasm from other pigmented lesions, including pigmented (melanotic) neurofibroma (PMN), psammomatous melanotic schwannoma (PMS), neurocristic cutaneous hamartoma (NCH), and desmoplastic malignant melanoma (DMM). We would like to stress that surgical pathologists and dermatopathologists need to be aware of the prototypical histological appearance of BT as there is the risk of misdiagnosing it either as pigmented tumors associated with neurocutaneous syndromes, such as PMN and PMS, or as a highly malignant melanocytic neoplasm (DMM).

Language: English

Type (Professor's evaluation): Scientific

No. of pages: 6