(Last Updated On: May 4, 2019)
Authors: Aileen Christine N. Barreiro, MD, FPDS,  Johannes F. Dayrit, MD, FPDS,  Gabriel R. Natividad, MD, FPCS

Abstract

Introduction. 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 sarcomas1.

 

Case Summary. 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 spindly 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.

 

Conclusion. 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 of with adequate margins will prevent recurrence of the tumor.

 

Citation

Barreiro AC, Dayrit J, Natividad G. Dermatofibrosarcoma protuberans on the leg: pearls in diagnosis and surgical management. J Phil Dermatol Soc. 2018; 27(1): 84-6.

 

Keywords

dermatofibrosarcoma, tumor, malignancy.

  1. Chen, Chih-Shan Jason, MD, PhD. (2010) Dermatofibrosarcoma protuberans. Retrieved December 23, 2011 from http://emedicine.medscape.com/article/1100203-overview#a0199
  2. Weiss S, Goldblum JR. Fibrohistiocytic Tumors of Intermediate Malignancy. In: Enzinger & Weiss’s Soft Tissue Tumors 5th Ed., China: Mosby Elsevier, 2008; pp. 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: Lippincott Williams & Wilkins, 2005; pp. 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 Genet Cytogenet, 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 imatinib mesylate, 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 Cancer Soft 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.

 

More Articles

A randomized, double-blind, comparative study on the safety and efficacy of virgin coconut (Cocos nucifera l.) oil against 1% hydrocortisone lotion as an anti-inflammatory and antipruritic preparation for mosquito reactions

  Authors: Uy, Veronica S, MD; Gracia B. Teodosio, MD, FPDS; Ma. Teresita G. Gabriel, MD, FPDS; Mary Catherine T. Galang, MD; Mohammad Yoga A. Waskito, MD; Johannes F. Dayrit, MD, FPDS     Abstract Background: Virgin coconut oil (VCO) has been reported...

read more