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Dermatofibrosarcoma Protuberans
Diagnosis
DFSP initially presents as asymptomatic, indolent, indurated plaque or firm, irregular cutaneous nodule(s), varying in color from flesh to reddish brown and with a slow increase in size of the sarcoma. After several years, protuberant nodules develop within the plaque, and the growth rate increases. Approximately 25% of patients complain of ulceration, bleeding, or pain at the lesion site.1,2 The lesions most commonly occur on the trunk (50%-60%), followed by the proximal extremities (20%-30%), and the head and neck (10%-15%).1-3
Histologically, DFSP resembles a well-differentiated fibrosarcoma. In the plaque type of DFSP, slender tumor cells with large, spindle-shaped nuclei embed uniformly in the collagen stroma, parallel to the skin surface. Mitotic figures are sparse.3-5 In the nodular type, dense, uniform proliferation of fibroblastic tumor cells and irregular, short, intersecting bands of tumor cells form a storiform pattern. Also typical are cells radiating from a central hub of fibrous tissue, forming a cartwheel pattern.3
Macroscopic and microscopic presentation of DFSP.
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| A. Solitary masses of subcutis and skin, average size 5 cm. |
B. May lead to ulceration of overlying skin with associated hemorrhage and cystic changes. |
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| C. Cellular tumors of dermis and subcutis; slender fibroblasts in monotonous storiform pattern. |
D. Little pleomorphism with low to moderate mitotic activity. |
From: www2.uic.edu/stud_orgs/prof/medclass2008/ pathslides/softtissuepathology.ppt - and
http://www.uphs.upenn.edu/path/LabServices/cytowebcase/cyto014.html
References:
- Stojadinovic A, Karpoff HM, Antonescu CR, et al. Dermatofibrosarcoma protuberans of the head and neck. Ann Surg Oncol. 2000;7:696-704.
- McArthur G. Molecularly targeted treatment for dermatofibrosarcoma protuberans. Semin Oncol. 2004;31:30-36.
- Gloster HM, Jr. Dermatofibrosarcoma protuberans. J Am Acad Dermatol. 1996;35:355-374; quiz 375-376.
- Martin L, Piette F, Blanc P, et al. Clinical variants of the preprotuberant stage of dermatofibrosarcoma protuberans. Br J Dermatol. 2005;153:932-936.
- Sabater-Marco V, Perez-Valles A, Berzal-Cantalejo F, et al. Sclerosing dermatofibrosarcoma protuberans (DFSP): an unusual variant with focus on the histopathologic differential diagnosis. Int J Dermatol. 2006;45:59-62.
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