Variability in Presentation of Dermatofibrosarcoma Protuberance: A Reterospective Review From Single Center

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Author(s): Dr. Sobia Manzoor

Received: 11/01/2021 | Revised: 29/01/2022 | Published: 29/01/2022 | Pages: 12-20 | DOI: 10.6007/PJPS/v10-i1/4

Background: Dermatofibrosarcoma protuberance(DFSP) is slow growing, locally aggressive tumor of skin and subcutaneous tissue. It has multiple variants which show different clinical features and malignant potential. The aim of this retrospective review is to compare variation in presentation and management of DFSP.
Objective: To better understand the variability in clinical presentation of dermatofibrosarcoma protuberance (DFSP) in patients treated at Shaukat Khanum Memorial Hospital & Research Center. Methodology: We retrieved data, of patients with DFSP who underwent surgery from December 2014 to December 2020, from hospital data base system. Information about patient’s demographics, clinical features, surgical treatment, complications and outcome was and collected on proforma.
Results: A total of 63 patients presented with DFSP with mean(SD) age 38.56(12.1) years, of which 69.8%(44) were males in their 3rd and 4th decade. Most common site was trunk in 41.3%(26). Most common tumor appearance was nodular in O-DFSP i.e. 26(49.1%) and 3(30%) in FS-DFSP group (p=0.03). Most of O-DFSP patients (84.9%) had size <10cm while more FS-DFSP patients (70%) had tumor size >10cm (p=0.0001). FS-DFSP patients were more prone to develop post-operative complications. Most of the patients of both groups are alive without disease i.e, 60% of FS-DFSP group and 54.7% of O-DFSP group(p=0.05).
Conclusion: Clinical characteristics of O- DFSP are non-specific and variable mimicking benign lesions. Short duration, ulcerated lesion with discharge, enlarged regional lymph nodes, and local recurrence should raise suspicion of FS-DFSP. Long-term follow-up is strongly recommended.

Keywords | Dermatofibrosarcoma protuberance, O-DFSP, FS-DFSP, Wide excision, Recurrence, Radiotherapy.