Dermatofibrosarcoma with multiple recurrences revisited a case series with review of literature

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Author(s): Rosheen Zahid, Waqas Mughal, Asif Bhatti, Usman sarwar, Ahmed shahzad 1-5 Shalamar Medical College, Lahore.

Received: 16/09/2022 | Published: 31/10/2023 | Pages: 66-71 |


Background:

Dermatofibrosarcoma protuberans (DFSP) is a rare, low to moderate grade sarcoma with protuberans-like growths. It usually starts from the dermis and invades the subcutaneous tissue, primarily on the trunk and limbs. It frequently occurs locally. It is uncommon for distant metastasis to occur. Common lesions are initially assumed to lead to misdiagnosis due to the superficial look of DFSP. Imaging techniques are not always used, and their correct management is delayed.

Material and Methods: A retrospective study of six patients with recurrent DFSP treated at our institution during the previous four years was conducted, with a focus on disease outcome, recurrence rates and during follow up disease-free intervals.

Results: Mean age was 37.5 years. Male to female ratio was 1:2. The tumor size ranged from 5 to 15 cm. treatment included local excision with post op radiotherapy in four patients and excision alone in 2 patients. All the patients remained disease-free in the follow-up period.

Conclusion: Vigorous, surgical resection with disease-free margins and post op management with or without radiation, minimizes recurrence and offer good results. As late recurrences may occur, literature supports that all patients with DFSP should be observed for more than 5-yrs.

KEYWORDS: DFSP, wide local excision, Soft tissue sarcoma and Dermal sarcoma,