2012.3-23.Blehoslav Bednar

DOI: 10.7241/ourd.20123.59                                                                                  article in PDF
Our Dermatol Online. 2012; 3(3): 239-240
Date of submission: 26.02.2012 / acceptance: 16.03.2012
Conflicts of interest: None

Khalid Al Aboud

Pathology Department, Wake Forest University, Winston-Salem, NC, USA

Corresponding author: Dr. Khalid Al Aboud     e-mail:amoa65@hotmail.com



Dermatofibrosarcoma protuberans (DFSP) a locally aggressive soft tissue neoplasm with intermediate- to low-grade malignancy [1]. It is reported in the literature as early as 1890. Darier and Ferrand first described it in 1924 as a distinct cutaneous disease entity called progressive and recurring dermatofibroma. Hoffman officially coined the term dermatofibrosarcoma protuberans in 1925 [1]. Pathologically this tumor is characterized in its early stage by large, spindle-shaped nuclei which are embedded fairly uniformly in the collagen stroma, parallel to the skin surface and infiltrating into the subcutaneous tissue in the honeycomb pattern. There is absence of cellular atypia, and mitoses are rare. In the nodular stage, there are irregular, short, intersecting bands of tumor cells forming a storiform pattern. Also typical are cells radiating from a central hub of fibrous tissue forming a cartwheel pattern. Occasionally, DFSP may show focal fibrosarcomatous changes with a characteristic herringbone pattern [1].The tumor is diffusely positive for CD34. There are several histological variants of DFSP. These include Bednar tumor, fibrosarcomatous, fibrosarcomatous with myoid/myofibroblastic change, myxoid,granular cell, palisaded, giant cell fibroblastoma, combined and indeterminate [2,3]. Pigmented DFSP (Bednar tumor) is morphologically and clinically identical to ordinary DFSP, with the exception of the presence of non-neoplastic melanin-laden dendritic cells which usually found scattered between the neoplastic spindleshaped cells. It is thought to represent the colonization of DFSP by melanocytes [2]. It constitutes 5%-10% of all cases of DFSP and shows morphologic features that overlap with melanocytic and fibrous proliferations [2-6]. There is one report each of a recurrent DFSP transforming into a Bednar tumor, Bednar tumor with prominent meningothelial-like whorls [4], Bednar tumor with dermal melancytosis, and congenital Bednar tumor in a patient with Fanconi anemia [6]. Rare cases occur with histological overlap between a pigmented dermatofibroma and pigmented DFSP [2]. Bednar tumor has, also, been reported at the site of previous immunization. There is no metastasis reported with this type of DFSP [2]. Pigmented DFSP is first reported by Bednar in 1957 [7], while describing a group of nine cutaneous tumors characterized by indolent growth and a prominent storiform pattern and in four cases by the presence of melanin pigment. He regarded these tumors as variants of neurofibroma (storiform neurofibroma) [3]. Blahoslav Bednar (1916-1998), is a well-known Czech pathologist [8-10] (Fig. 1). The Correct spelling for his name in Czech is, Blahoslav Bednáø. He founded the official journal Czecho-Slovak Pathology, and he modernized Hlava Institute of Pathology [10]. Bednar made a great contribution to the pathology and he left behind hundreds of interesting publications and researches.

Figure 1. Blahoslav Bednar (1916-1998). A courtesy of Jaromir Hacek, Editor of Cesko-slovenska patologie Journal. 
The reprint of this figure is also approved by the committe of the Czech Society of Pathologists
1. Chen CJ: Dermatofibrosarcoma Protuberans. E-Medicine online dermatology textbook (www.emedicine.com), Copyright © 1994-2012 by WebMD LLC. Published, 2001. Available on line at; http://emedicine.medscape.com/article/1100203-overview 2. Weedon D: Dermatofibrosarcoma protuberance.Chapter34, Tumors and tumor-like proliferations of fibrous and related tissues.Weedon’s skin pathology. 3ed edn. 2010. Elsevier limited. P809-844. 3. Weiss SW,Goldblum JR: Bednar tumor (pigmented dermatofibrosarcoma protuberance, storiform neurofibroma). Chapter 13. Fibrohistiocytic tumors of intermediate malignancy. Weiss & Goldblum: Enzinger and Weiss’s Soft Tissue Tumors, 5th ed. 2008, Mosby, Inc. P371-402. 4. Wang J, Yang W: Pigmented dermatofibrosarcoma protuberans with prominent meningothelial-like whorls. J Cutan Pathol. 2008; 35, Suppl 1: 65-69. 5. McAllister JC, Recht B, Hoffman TE, Sundram UN: CD34+ pigmented fibrous proliferations: the morphologic overlap between pigmented dermatofibromas and Bednar tumors. Am J Dermatopathol. 2008; 30: 484-487. 6. Lee DW, Yang JH, Won CH, Chang SE, Lee MW, Choi JH, et al: A case of congenital pigmented dermatofibrosarcoma protuberans (Bednar tumor) in a patient with Fanconi anemia. Pediatr Dermatol. 2011; 28: 583-585. 7. Bednar B: Storiform neurofibromas of the skin, pigmented and nonpigmented. Cancer. 1957, 10: 368-376. 8. Dobiás: [The 60th birthday of Prof. B. Bednár]. Cesk Patol. 1976; 12: 202: 208. 9. Trapl J: [The 60th birthday of Prof. Bednár, 18 December 1916]. Cas Lek Cesk. 1976; 115: 1560. 10. Blahoslav Bednar.[a page on the internet]. from ,who named it? © 1994 – 2012 Ole Daniel Enersen. Last modified, not mentioned. Cited on Feb 20, 202.Available on; http://www.whonamedit.com/doctor.cfm/3270.html



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