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.
- Hayakawa K , Matsumoto S, Ae K, et al. Risk factors for distant metastasis of dermatofibrosarcoma protuberans. J Orthop Traumatol. 2016 Sep; 17(3): 261–266.
- Li Y, Liang J, Xu X, Jiang X, Wang C, Chen S, et al. Clinicopathological features of fibrosarcomatous Dermatofibrosarcoma Protuberans and the construction of a back-propagation neural network recognition model. Orphanet J Rare Dis 2021; 16(48): 1-9.
- Chiang K.T, Lee S.Y, Chu H. Dermatofibrosarcoma protuberans, a rare but locally aggressive tumor on finger: clinical and aeromedical considerations. Case Reports Plast Surg Hand Surg. 2015; 2(1): 15–18.
- Rasheed AA, Barwad A, Dhamija E, Garg R, Pandey R, Shamim SA, et al, Advanced dermatofibrosarcoma protuberans: an updated analysis of cases from an Indian sarcoma clinic. Future Science OA 2021; 7(9):1-11.
- Li Y , Wang C, Xiang B, et al. Clinical Features, Pathological Findings and Treatment of Recurrent Dermatofibrosarcoma Protuberans. J Cancer. 2017; 8(7): 1319–1323.
- Huis In ‘t Veld EA, van Coevorden F, Grunhagen DJ, Smith MJ, van Akkooi ACJ, Wouters M, et al. Outcome after surgical treatment of dermatofibrosarcoma protuberans: is clinical follow-up always indicated? Cancer. 2019;125(5):735–41.
- Hao X, Billings S.D, Wu F, et al.Dermatofibrosarcoma Protuberance: Update on the Diagnosis and Treatment. J Clin Med. 2020 Jun; 9(6): 1752.
- Ahmad I, Mir MA, Bariar LM, Afroz N. Recurrent Dermatofibrosarcoma Protuberance and its Management with Radical Excision and Interval Skin Grafting: A case Report. World J Plast Surg 2016;5(1):67-71.
- Stacchiotti S, Astolfi A, Gronchi A, et al. Evolution of Dermatofibrosarcoma Protuberans
to DFSP-Derived Fibrosarcoma: An Event Marked by Epithelial–Mesenchymal Transition–like Process and 22q Loss. Mol Cancer Res 2016;14:820-829. - Du K, Li J, Tang L, Lin X, Kong X, Liao X, et al. Role of postoperative radiotherapy in dermatofibrosarcoma protuberans: a propensity score-matched analysis. Radiat Oncol. 2019;14(20):1-8.
- Effiom OA, Olojede ACO, Akinde OR, Olawuyi AB, Amoo AT, Arotiba GT. Dermatofibrosarcoma Protuberans: clinicopathologic presentation in Nigerians. Pan Afr Med J. 2018; 31(25): 1-7.
- Li Y, Wang C, Xiang B, Chen S, Li L, Ji Y. Clinical Features, Pathological Findings and Treatment of Recurrent Dermatofibrosarcoma Protuberans. J Cancer. 2017 May 12;8(7):1319-1323.
- Larbcharoensub N, Kayankarnnavee J, Sanpaphant S, et al. Clinicopathological features of dermatofibrosarcoma protuberans. Oncol Lett. 2016 Jan;11(1):661-667.
- Kashyap L, Noronha V, Patil V, et al. Dermatofibrosarcoma protuberans of head and neck: Clinical outcome of nine cases treated with imatinib. Cancer Res Stat Treat 2019;2(1):112-8.
- Yadav S, Verma N, Khurana N, Neogi S. Recurrent DermatofibrosarcomaProtuberanswith Pigmentation and Myoid Differentiation. Sultan Qaboos Univ Med J. 2018 May;18(2):e228-e230.
- Kim BJ, Kim H, Jin US, et al. Wide Local Excision for Dermatofibrosarcoma Protuberans: A Single-Center Series of 90 Patients. Biomed Res Int. 2015; 2015: 642549.
- Ramirez-Fort MK, Meier-Schiesser B, Niaz MJ, et al. Dermatofibrosarcoma Protuberans: The Current State of Multidisciplinary Management. 2020;18(5):288-293.
- Rutkowski P, Debiec-RychterM. Current treatment options for dermatofibrosarcoma protuberans. Expert Rev Anticancer Ther 2015;15(1):901–9.
- Liang CA, Jambusaria-Pahlajani A, Karia PS, et al. A systematic review of outcome data for dermatofibrosarcoma protuberans with and without fibrosarcomatous change. J Am Acad Dermatol 2014;71(1):781-
- Nardelli AR, Marcarini R, De Franco MFB, et al. Dermatofibrosarcoma protuberans rapid growth after incisional biopsy. Med Cutan Iber Lat Am. 2017;45(1):41-44.
- Saiga P, Grob JJ, Lebbe C, et al. Diagnosis and treatment of dermatofibrosarcoma protuberans. European consensus-based interdisciplinary guideline. Eur J Cancer 2015 Nov; 51 (17): 2604-8.
- Brooks J. Dermatofibrosarcoma protuberans. [Updated 2022January, Cited 2022 March]
Available from: [https://www.ncbi.nlm.nih.gov/books/NBK513305/]
All published material becomes the sole property of the Pakistan Journal of Plastic Surgery, copyrighted by the Pakistan Association of Plastic Surgeons (PAPS). By submitting an article or brief communication, all authors agree to these conditions.
During the submission process, all authors have required to complete a copyright transfer agreement, “Author Consent form” By completing the copyright transfer agreement, you are transferring copyright of figures, tables, videos, and the content of the manuscript to the Pakistan Association of Plastic Surgeons (PAPS).
If you do not wish to or cannot transfer copyright of some or all of your content, please contact the editorial office at pjpspakistan@gmail.com