Introduction And Importance: Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma affects mainly the trunk and proximal limbs. Clinically, it typically presents as an asymptomatic plaque or nodular-like lesion that progresses slowly before entering a rapid growth phase. DFSP exhibits a low potential for metastasis, mainly in cases where fibrosarcomatous transformation occurs, but it has a high rate of local recurrence. Diagnosis of DFSP is often delayed, and it is challenging to establish without performing a biopsy and histologic analysis. The mainstay treatment for DFSP is surgical wide excision with free margins, although this can be challenging depending on the location of the tumor.
Case Presentation: We report a rare presentantion of dermatofibrosarcoma protuberans according to the SCARE guidelines. The patients main concern was the slow evolving mass on the dorsum of the foot that at presentation was 1x1cm. The biopsy showed a dermatofibrosarcoma protuberans. A radical excision involving ray amputation of the 2nd and 3rd finger provided a 1 cm clear margin. CLíNICAL DISCUSSION AND CONCLUSION: This case shows how an inconspicuous nodule in an uncommon area can be the primary manifestation of a serious condition.
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| http://dx.doi.org/10.1016/j.ijscr.2023.108667 | DOI Listing |
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Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade cutaneous sarcoma typically found on the proximal extremities and the trunk, characterized by infiltrative growth and low risk of metastasis. High rates of local recurrence or relatively large tumor sizes can significantly complicate therapeutic management, particularly when 1) surgical intervention is not adequately performed and /or 2 access to newer medications is limited or their high cost imposes a financial burden on patients. We present the case of a 63-year-old male with a histologically confirmed dermatofibrosarcoma protuberans, measuring 6 cm in diameter, located on the right dorsal region, accompanied by several confluent multifocal nodules situated infralaterally to the primary formation.
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