dermatofibrosarcoma protuberans dfsp with fibrosarcomatous transformation a case report
2021
Abstract Case Report: We report a case of a 40 year-old woman, who consulted for an infraumbilical 5cm 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 histopatological 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
(10-60%), being weird lymphatic dissemination and distant hematogenous metastasis (4-5%). 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 (7-16% 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
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