Case Report Dermatology 1992;184:281-282

J.I. López. J.M. Elizalde A. Fdez-Larrinoa

Pigmented Dermatofibrosarcoma protuberans (Bednar Tumour)

Servicio de Anatomía Patológica, Hospital Civil de Bilbao. España

Key Words Pigmented dcrmatofibrosarcoma protuberans Bednar tumour Immunohistochcmistry Histogenesis

Abstract A case of pigmented dermatofibrosarcoma protuberans in a 37-year-old man is reported. The tumour presented as a nodular lesion located in the left scapular area. Surgical resection with wide margins was performed. No recurrences have appeared to date. The presumptive origin of this entity is briefly discussed. ............................

Introduction The pigmented variant of dermatofibrosarcoma protu­ berans (Bednar tumour) is a rare entity, for only approxi­ mately 45 cases have been previously reported in the inter­ national literature [1 ]. A new case is reported herein. The origin as well as the clinical, light-microscopic and immuno­ histochemical features of this entity are briefly discussed.

Case Report

Received: September 30. 1991 Accepted: November 26, 1991

Fig. 1. Panoramic view of a dense proliferation of spindle cells arranged in a storiform pattern. HE. x76.

cells, suggesting that the pigment was melanin. By immunohistochcm­ istry. vimentin (V9; Dakopattsl was strongly positive in proliferating cells. S-lOO protein (polyclonal; Dakopatts) only stained dendritic pig­ ments cells. Finally, HMB-45 (Enzo) - a 10-kD polypeptide that rec­ ognizes both benign and malignant melanocytic proliferations |2. 3] - antibody immunostain w'as negative. One year later, no recurrences have appeared.

Dr. Jose I. Lope/ Servicio de Anatomia Patologica Hospital Civil de Bilbao Avda. de Montevideo IX. E-48013 Bilbao (Spain)

© 1992 Karger A (i. Basel 1018-8665/92/1844-0281 $ 2.75/0

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A 37-year old man consulted for a skin nodule, measuring approxi­ mately 4 cm in diameter, on his left scapular region. The tumour had grown rapidly during the previous months and. on examination, had an elastic consistency. Neither ulceration nor other secondary changes were observed in the overlying epidermis. The lesion was resected with wide surgical margins. Macroscopic section showed a whitish lesion with imprecise bor­ ders that mainly affected the subcutis. Histopathologically, a dense cellular proliferation without well-defined margins was evidenced. Proliferating cells displayed a fibroblastic appearance and were arranged in a typical storiform pattern (fig. 1). Cellular anaplasia was mild, and mitoses were only occasionally seen (less than 1/10 highpower fields). Remarkably, some highly pigmented and odd-shaped cells were randomly distributed in the neoplastic growth (fig. 2). Masson-Fontana stain was strongly positive in these dendritic-appearing

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Discussion **

Bednar tumour was first recognized as a specific variant of dermatofibrosarcoma protuberans in 1957 [4], and since then, a small number of isolated cases and short series have been published [1,5-12]. Despite its rarity, its clinical and pathologic features are well established [13, 14], The bio­ logical behaviour seems to be similar to dermatofibrosar­ coma protuberans. although local recurrences have been less commonly observed in Bednar tumour [ 1]. This fact is presumptively related to incomplete surgical resections [ 10, 12, 13]. Distant métastasés have never been well docu­ mented to date. As different origins have been proposed, Bednar tumour has received several names [4, 5, 7. 9, 10]. Although still not clear, the presence of pigmented, mclanosome-containing cells has been considered a strong evi­ dence of a neural crest origin [10. 11], However, it has also been proposed that the intralesional presence of these cells is the consequence of a secondary colonization in a pre­ vious non-pigmented dermatofibrosarcoma protuberans [12], As for other entities secondarily colonized by nonncoplastic melanocytes [12. 15]. a hypothetical breach in the overlying epidermal basement membrane could also be

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i 1 Fig. 2. Dendritic pigmented cells stained positively by the Masson-Fontana method. x305.

the causative factor in developing Bednar tumour. The fibroblastic nature of most cells, fairly demonstrated by ultrastructural and immunohistochemical methods [1, 10-12], strongly supports an origin similar to that of the ordinary dermatofibrosarcoma protuberans.

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DingJ. Hashimoto H.SugimotoT.Tsuneyoshi M. Enjoji M: Bednar tumor (pigmented der­ matofibrosarcoma protuberans). An analysis of six cases. Acta Pathol Jpn 1990:40:744-754. Wick MR, Swanson PE. Rocamora A: Recog­ nition of malignant melanoma by monoclonal antibody HMB-45. An immunohistochemical study of 200 paraffin-embedded cutaneous tumors. J Cutan Pathol 1988:15:201-207. Smollcr BR. McNutt NS. Hsu A: HMB-45 rec­ ognizes stimulated melanocytes. J Cutan Pathol 1989:16:49-53. Bednar B: Storiform neurofibromas of the skin, pigmented and nonpigmented. Cancer 1957:10:368-376. Bednar B: Storiform neurofibroma in the core of naevocellular naevi. J Pathol 1970:101: 199-201. Hashimoto K. Brownstein MH. Jakobicc FA: Dermatofibrosarcoma protuberans. A tumor with perineural and endoneural cell features. Arch Dermatol 1974:110:874-885. Nishio K. Koda H : A case of storiform neurofi­ broma. J Dermatol (Tokyo) 1975:2:143-148.

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Santa Cruz DJ. Yates AJ: Pigmented storiform neurofibroma. J Cutan Pathol 1977:4:9-13. 9 Miyamoto Y. Morimatsu M. Nakashima T: Pigmented storiform neurofibroma. Acta Pathol Jpn 1984:34:821-826. 10 Dupree WB. Langloss JM. Weiss SW: Pig­ mented dermatofibrosarcoma protuberans (Bednar tumor). A pathologicv. ultrastruc­ tural. and immunohistochemical study. Am J Surg Pathol 1985:9:630-639. 11 Nakamura T. Ogata H. Katsuyama T: Pig­ mented dermatofibrosarcoma protuberans. Report of two cases as a variant of dermatofi­ brosarcoma protuberans with partial neural differentiation. Am J Dermatopatlrol 1987:9: 18-25. 12 Fletcher CDM. Theaker JM. Flanagan A. Krausz T: Pigmented dermatofibrosarcoma protuberans (Bednar tumour): Melanocytic colonization or neuroectodermal differentia­ tion? A clinicopathological and immunohisto­ chemical study. Histopathology 1988:13: 631-643.

13 Enztnger FM. Weiss SW: Fibrohistiocytic tumors of intermediate malignancy: in Enzingcr FM and Weiss SW (ed): Soft Tissue Tumors, ed 2. St Louis. Mosby. 1988. pp 252-268. 14 Lever WF. Schaumburg-Lever G: Tumors of fibrous tissue: in Lever WF and SchaumburgLever G (ed): Histopathology of the Skin, ed 7. Philadelphia. Lippincott. 1990. pp 673-674. 15 Azzopardi JG. Euscbi V: Melanocyte coloniza­ tion and pigmentation of breast carcinoma. Histopathology 1977:1:21-30.

Lopez/F.lizaldc/Fdez-Larrinoa

Bednar Tumour

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References

Pigmented dermatofibrosarcoma protuberans (Bednar tumour).

A case of pigmented dermatofibrosarcoma protuberans in a 37-year-old man is reported. The tumour presented as a nodular lesion located in the left sca...
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