Dermatofibrosarcoma protuberance: a recrudescent dermal malignancy
DOI:
https://doi.org/10.18203/2349-2902.isj20202865Keywords:
Dermatofibrosarcoma protruberens, Sarcoma, Wide local excision, Imatinib, Spindle cells, Storiform patternAbstract
Dermatofibrosarcoma protruberens is a rare soft tissue tumour of low malignant potential, commonly seen on the trunk and extremities. It is a slow growing sarcoma, with a tendency for recurrence. Rarely, it may be a high-grade tumour. It responds well to wide local excision. Radiotherapy is beneficial for margin positive and high-grade tumours. We present a case of a 22 years old male with a dermatofibrosarcoma protruberens presenting as a swelling over the left inguinal region since, 4 years, that was excised with a 2cm margin. Patient was followed up for six months without any recurrence.
References
Vita DVT, Hellman S, Rosenberg SA, eds. Cancer: Principles and Practice of Oncology. 6th ed. Philadelphia, PA: Lippincott Williams and Wilkins; 2001:1841-1891.
Rutgers EJ. Dermatofibrosarcoma protuberans: treatment and prognosis. European J Surg Oncology. 1992;18(3):241-8.
Doreen L. Current treatment options in dermatofibrosarcoma protuberans. J Cancer Res Clin Oncology. 2009;135(5):653-65.
Mastrangelo, Giuseppe. Incidence of soft tissue sarcoma and beyond: a population‐based prospective study in 3 European regions. Cancer. 2012;118(21):5339-48.
Jemal A, Tiwari RC, Murray T. Cancer statistics, 2004. CA Cancer J Clin. 2004;54:8-29.
Suit H, Spiro I, Mankin HJ, Efird J, Rosenberg AE. Radiation in management of patients with dermatofibrosarcoma protuberans. J Clin Oncol. 1996;14:2365-9.
Hoffmann E. Ueber das knollentribende Fibrosarkom der Haut (dermatofibrosarcoma protruberans).
Lindner NJ, Scarborough MT, Powell GJ, Spanier S, Enneking WF. Revision surgery in dermatofibrosarcoma protuberans of the trunk and extremities. Eur J Surg Oncol. 1999;25:392-7.
Pack GT, Tabah EJ. Dermatofibrosarcoma protruberans: a report of 39 cases. Arch Surg. 1951;62:391-411.
Torreggiani WC, Ismail AK, Munk PL, Nicolaou S, Connell OJX, Knowling MA. Dermatofibrosarcoma protuberans: MR imaging features. AJR Am J Roentgenol. 2002;178:989-93.
Bae SH, Lee JY. Imaging Features of Breast Dermatofibrosarcoma Protuberans in Various Modalities Including FDG-PET CT. Iran J Radiol. 2016;13(2):33916.
Kocjan, Gabrijela. Diagnostic Dilemmas in FNAC Cytology: Soft-Tissue Lesions. Fine Needle Aspiration Cytology: Diagnostic Principles and Dilemmas; 2006: 151-180.
Chang CK, Jacobs IA, Salti GI. Outcomes of surgery for dermatofibrosarcoma protuberans. European J Surg Oncology. 2004;30(3):341-5.
Lindner NJ. Revision surgery in dermatofibrosarcoma protuberans of the trunk and extremities. European J Surg Oncology. 1999;25(4):392-7.
Ballo MT, Zagars GK, Pisters P, Pollack A. The role of radiation therapy in the management of dermatofibrosarcoma protuberans. Int J Radiat Oncol Biol Phys. 1998;40:823-7.
Tazzari, Marcella. Adaptive immunity in fibrosarcomatous dermatofibrosarcoma protuberans and response to imatinib treatment. J Investigative Dermatol. 2017;137(2):484-93.
Ugurel S, Mentzel T, Utikal J, Helmbold P, Peter M, Pfohler C, et al. Neoadjuvant imatinib in advanced primary or locally recurrent dermatofibrosarcoma protuberans: a multicenter phase II DeCOG trial with long-term follow-up. Clin Cancer Res. 2014;20(2):499-510.
