Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma of the skin characterized by plaque like tumor derived from mesenchymal cell. Fibrosarcomatous transformation of DFSP (FS-DFSP) is an even rarer variant of which it is clinically indistinguishable from other DFSP variant – diagnosed by histopathological characteristics. As it is known to be more aggressive with higher risk of distant metastases, treatment should be carefully planned followed by strict post-op follow ups.
This is a case of a 57 years old man with a slow growing large tumor on his forearm following a minor trauma 10 years ago, presented to our institution for further management due sudden rapid enlargement and discomfort. The result of the lesion sent for histopathological examination is diagnosed as FS-DFSP without distant metastases (from a full body CT scan). On 3rd month of follow up, neither recurrence nor symptoms of distant metastases has been observed.
In conclusion DFSP needs a histopathological examination to distinguish its variant. This is important as FS-DFSP has higher risk of recurrence and metastasis. There are options of technique to ensure complete surgical resection of tumor. In cases of metastases and unresectable tumor, radiotherapy and pharmacological treatment is to be considered. In our case, there is no signs of recurrent or distant metastases. However, long -term follow up is strictly recommended.