Site Under Construction

Dermatofibrosarcoma protuberans on the leg: pearls in diagnosis and surgical management

Aileen Christine N. Barreiro, MD, FPDS, Johannes F. Dayrit, MD, FPDS, Gabriel R. Natividad, MD, FPCS



Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing soft tissue tumor accounting for less than 0.1% of all malignant neoplasms and approximately 1% of all soft tissue sarcomas.



We report a case of a 27-year-old female who presented with a 1-year history of a gradually enlarging firm, erythematous to violaceous nodule on the right anterolateral leg. Shave excision biopsy was done, and revealed spindle-shaped fibroblasts arranged in a storiform pattern around an indistinct vasculature. Histopathologic diagnosis revealed dermatofibrosarcoma protuberans. There was prominent, diffuse, positivity for CD34 by immunohistochemistry. The cytogenetic analysis revealed a t(17;22) translocation, confirming our working impression.The patient underwent wide excision with frozen section. All surgical margins of resection were negative for tumor. The patient was referred to plastic surgery for post-excision coverage defect. Split-thickness skin graft was applied over the defect. Patient tolerated the procedure well, and has not experienced recurrence of the tumor.



A protuberant, well-circumscribed tumor that seems benign may incidentally be a sarcoma. Skin biopsy, immunohistochemistry and cytogenetic studies using Fluorescence In-Situ Hybridization (FISH) are the most essential laboratory investigations to validate a diagnosis of DFSP. Proper surgical excision with adequate margins will prevent recurrence of the tumor.


  1. Chen, Chih-Shan Jason, MD, PhD. (2010) Dermatofibrosarcoma protuberans. Retrieved December 23, 2011 from
  2. Weiss S, Goldblum JR. Fibrohistiocytic Tumors of Intermediate Malignancy. In: Enzinger & Weiss’s Soft Tissue Tumors 5th Ed., China: Mosby Elsevier, 2008; p. 371-382.
  3. Ten Heuvel, S. Dermatofibrosarcoma protuberans: Recurrence is related to the adequacy of surgical margins, Eur J Surg Oncol, 2001; 36(1), pp. 89-94
  4. Heenan, Peter J. Tumors of Fibrous Tissue Involving the Skin. In: Elder DE, Elenitsas R, Johnson BL, Murphy GF, MD. Lever’s Histopathology of the Skin, Philadelphia: Lipincott Williams & Wilkins, 2005; p.984-986
  5. Rosai, Juan, MD. Skin-Tumors and tumor-like conditions. In:Rosai& Ackerman’s Surgical Pathology 9th Ed, China: Mosby Elsevier, 2011;pp. 1829-1834.
  6. Sandberg, Avery A., Bridge, Julia A. (2002) Updates on the cytogenetics and molecular genetics of bone and soft tissue tumors: dermatofibrosarcoma protuberans and giant cell fibroblastoma, Cancer GenetCytogenet,2003;140,1-12
  7. Labropoulos, S. V., Papadopoulos, S., Hadjiyiassemi, L. , Giannopoulou, D., Razis, D., Razis, E. D. Response of metastatic dermatofibrosarcoma protuberans to imatinibmesylate, J Clin Oncol,2003; 22
  8. Parker, Timothy, MD. Surgical Margins for Excision of Dermatofibrosarcoma protuberans, J Am Acad Dermatol, 1995; 32(233-6)
  9. Haas RL, Keus RB, Loftus BM, Rutgers EJ, van Coevorden F, Bartelink H. (1997) The role of radiotherapy in the local management of dermatofibrosarcoma protuberans, Eur J CancerSoft Tissue Tumours Working Group, 1997; 33(7):1055-60.
  10. Dagan R, Morris CG, Zlotecki RA, Scarborough MT, Mendenhall WM.Radiotherapy in the treatment of dermatofibrosarcoma protuberans, J Clin Oncol, 2005;28(6):537-9.
  11. Paradisi, A. (2008). Dermatofibrosarcoma protuberans: Wide local excision vs. Mohs micrographic surgery, Cancer Treat Rev, 2008; 34(8), pp. 728-736.

REquesting Permission

Dermatofibrosarcoma protuberans on the leg: pearls in diagnosis and surgical management