Dermatofibrosarcoma Protuberans (DFSP) with Fibrosarcomatous Transformation: A Case Report

Martínez MF, Vázquez MLF, Orue-Echebarría M, Román LM, Jiménez WV, Lominchar PL, García NP, Serrano FA, Martínez CP and Bayón LG*

Department of General Surgery, Hospital General Universitario Gregorio Marañón, Spain

^*Correspondance to: Luis González Bayón 

 PDF  Full Text DOI: 10.25107/2474-1647.3190

Abstract

Case Report: We report a case of a 40 year-old woman, who consulted for an infraumbilical 5 cm cutaneous node, the third recurrence of a fibrocystic lesion. An expert sarcoma pathologist analyzed the previous histopathological samples, and suspected a DFSP, finding a positive rearrangement for the PDGFB gene (derived growth factor beta polypeptide). Surgical removal with wide local excision was performed. The histopathological analysis showed a mesenchymal proliferation with storiform pattern according to a DFSP, and a fishbone pattern with fusiform cells corresponding to a fibrosarcoma in the deeper region. Backgrounds: DFSP is a rare soft tissue tumor affecting young adults, and has been related to a chromosomal translocation t(17;22) (q22;q13), presented in 90% of the cases, that results in a fusion protein COL1A1-PDGFB (collagen type 1A1). It is usually presented as an indurated skin plaque, involving dermis and subcutaneous fat. It has a high risk of local recurrence (10% to 60%), being weird lymphatic dissemination and distant hematogenous metastasis (4% to 5%). Histologically it is characteristic fusiform cells with a storiform pattern (cellular swirls). The treatment is surgical removal with wide local excision. It has a 10-year overall survival of 99%. It has been described de fibrosarcomatous degeneration (7% to 16% of the cases), with more aggressive behavior, a higher local recurrence and distant metastasis rate. Conclusion: A proper identification of this type of tumors may change the surgical procedure and disease prognosis, so they must be managed by expert pathologists and a multidisciplinary team on soft tissue sarcomas in order to achieve the best results.

Keywords

Dermatofibrosarcoma, Protuberans, Soft tissue; Fibrosarcoma

Cite the article

Martínez MF, Vázquez MLF, Orue-Echebarría M, Román LM, Jiménez WV, Lominchar PL, et al. Dermatofibrosarcoma Protuberans (DFSP) with Fibrosarcomatous Transformation: A Case Report. Clin Surg. 2021; 6: 3190..