DOI: http://dx.doi.org/10.18203/2349-2902.isj20203806

Large anterior abdominal wall fibrosarcoma in a known case of recurrent dermatofibrosarcoma protuberans

Snigdha Kamini, Sunil Kumar Jain, Jaspreet Bajwa, M. Nafees Ahamad, Sumit Kumar Prasad, Shivam Sharma, Pradeep Mekha Srinivas

Abstract


Dermatofibrosarcoma protuberans is a rare soft tissue sarcoma of low to intermediate malignant potential. Incidence is estimated to be 0.8 to 4.5 cases per million persons per year. The tumor is highly aggressive locally and is known to recur. Punch biopsy or excisional biopsy (in case of small tumors) are diagnostic. Computer tomography (CT) and magnetic resonance imaging (MRI) are useful in determining the size and extent of the tumor’s treatment is primarily surgical, with a wide local excision with at 2 cm margin. However, local recurrence after apparently adequate surgical excision is common. Mohs micrographic surgery has been recommended as it enables maximum preservation of tissue. When surgery is insufficient, imatinib mesylate is shown as a safe and effective treatment in dermatofibrosarcoma protuberans (DFSP), especially in cases of locally advanced or metastatic disease. Here, we report a case of a giant fibrosarcoma on the anterior abdominal wall, measuring 27×18×9 cm, which occurred in a recurrent dermatofibrosarcoma protuberans. Diagnosis was done by histopathological examination (HPE) of the previously excised tumor and CT was done. The tumor was excised with a 3 cm margin and extensive reconstruction of the anterior abdominal wall defect was done using synthetic mesh, myocutaneous flaps and split skin grafting. Keeping in view the recurrent nature of our case, large tumor size, DFSP-fibrosarcomatous (FS) transformation and close negative margins in the HPE of the postoperative specimen, the patient was planned for adjuvant radiotherapy.


Keywords


Dermatofibrosarcoma protuberans, Fibrosarcoma, Reconstruction

Full Text:

PDF

References


Criscito MC, Martires KJ, Stein JA. Prognostic factors, treatment, and survival in dermatofibrosarcoma protuberans. JAMA Dermatol 2016;152(12):1365-71.

Weinstein JM, Drolet BA, Esterly NB, Rogers M, Bauer BS, Wagner AM et al. Congenital dermatofibrosarcoma protuberans: variability in presentation. Arch Dermatology. 2003;139(2):207-11.

Llombart B, Serra-Guillén C, Monteagudo C, Guerrero JA, Sanmartín O. Dermatofibrosarcoma protuberans: a comprehensive review and update on diagnosis and management. InSeminars in diagnostic pathology. WB Saunders; 2013:30:13-28.

Rutkowski P, Przybył J, Świtaj T. Genetics of rare mesenchymal tumors: implications for targeted treatment in DFSP, ASPS, CCS, GCTB and PEComa. Int J Biochem Cell Biol. 2014;53:466-74.

Farma JM, Ammori JB, Zager JS, Marzban SS, Bui MM, Bichakjian CK et al. Dermatofibrosarcoma protuberans: how wide should we resect? Ann Surg Oncol. 2010;17(8):2112-8.

Roh MR, Bae B, Chung KY. Mohs’ micrographic surgery for dermatofibrosarcoma protuberans. Clin Exp Dermatol. 2010;35(8):849-52.

Rutkowski P, Dębiec‐Rychter M, Nowecki ZI, Michej W, Symonides M, Ptaszynski K et al. Treatment of advanced dermatofibrosarcoma protuberans with imatinib mesylate with or without surgical resection. J European Academy Dermatol Venereol. 2011;25(3):264-70.

Malhotra B, Schuetze SM. Dermatofibrosarcoma protruberans treatment with platelet-derived growth factor receptor inhibitor: a review of clinical trial results. Curr Opin Oncol. 2012;24(4):419-24.

Bowne WB, Antonescu CR, Leung DH, Katz SC, Hawkins WG, Woodruff JM et al. Dermatofibrosarcoma protuberans: a clinicopathologic analysis of patients treated and followed at a single institution. Cancer. 2000;88(12):2711-20.

Stacchiotti S, Pedeutour F, Negri T, Conca E, Marrari A, Palassini E et al. Dermatofibrosarcoma protuberans‐derived fibrosarcoma: Clinical history, biological profile and sensitivity to imatinib. International J Cancer. 2011;129(7):1761-72.

Pack GT, Tabah EJ. Dermatofibrosarcoma protuberans: a report of thirty-nine cases. Arch Surg. 1951;62(3):391-411.

Sun LM, Wang CJ, Huang CC, Leung SW, Chen HC, Fang FM et al. Dermatofibrosarcoma protuberans: treatment results of 35 cases. Radiotherapy and oncology. 2000;57(2):175-81.

Gloster JH, Harris KR, Roenigk RK. A comparison between Mohs micrographic surgery and wide surgical excision for the treatment of dermatofibrosarcoma protuberans. Journal of the American Academy Dermatol. 1996;35(1):82-7.

Rutgers EJ, Kroon BB, Albus-Lutter CE, Gortzak E. Dermatofibrosarcoma protuberans: treatment and prognosis. European J Surgical Oncol. 1992;18(3):241-8.

Loghdey MS, Varma S, Rajpara SM, Al-Rawi H, Perks G, Perkins W. Mohs micrographic surgery for dermatofibrosarcoma protuberans (DFSP): a single-centre series of 76 patients treated by frozen-section Mohs micrographic surgery with a review of the literature. J Plastic, Reconstructive Aesthetic Surg. 2014;67(10):1315-21.

Hamid R, Hafeez A, Darzi MA, Zaroo I, Rasool A, Rashid H. Outcome of wide local excision in dermatofibrosarcoma protuberans and use of radiotherapy for margin-positive disease. Indian Dermatol Online J. 2013;4(2):93.

Uysal B, Sager O, Gamsiz H, Cicek A, Demiral S, Dincoglan F et al. Evaluation of the role of radiotherapy in the management of dermatofibrosarcoma protuberans. J BUON. 2013;18(1):268-73.

Navarrete-Dechent C, Mori S, Barker CA, Dickson MA, Nehal KS. Imatinib treatment for locally advanced or metastatic dermatofibrosarcoma protuberans: a systematic review. JAMA Dermatol. 2019;155(3):361-9.

Cai H, Wang Y, Wu J, Shi Y. Dermatofibrosarcoma protuberans: clinical diagnoses and treatment results of 260 cases in China. Journal Surgical Oncol. 2012;105(2):142-8.