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      Call for Papers: Digital Platforms and Artificial Intelligence in Dementia

      Submit here by August 31, 2025

      About Dementia and Geriatric Cognitive Disorders: 2.2 Impact Factor I 4.7 CiteScore I 0.809 Scimago Journal & Country Rank (SJR)

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      Dermatofibrosarcoma Protuberans: A Study of 148 Patients

      brief-report

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          Abstract

          Introduction

          Dermatofibrosarcoma protuberans (DFSP) is the most common sarcoma of the skin. Although distant metastases are infrequent, DFSP is highly aggressive locally with frequent local recurrences. It has been reported that the presence within the tumour of areas histopathologically mimicking fibrosarcoma may increase the risk of recurrence.

          Objective

          The objective of this study was to review the clinical features of our patients with DFSP and the factors associated with recurrence of the tumour, focussing on the presence of fibrosarcomatous areas.

          Methods

          Retrospective study of patients with DFSP diagnosed in 1990–2021 in a tertiary university hospital. The medical records were reviewed to obtain the following data: age, sex, tumour location, diameter, evolution time, presence of fibrosarcomatous areas, development of recurrence, and follow-up. Factors possibly associated with disease-free survival were analysed with Kaplan-Meier method and multivariate Cox regression.

          Results

          148 patients (74 women/74 men, mean age 46.28 years, SD 14.431) were included in the study. Tumours involved the head and neck in 15 cases, thorax in 31, abdomen in 16, upper back in 43, lower back in 10, upper extremities in 10, and lower extremities in 23. Fibrosarcoma-like areas were observed in 16 tumours (10.81%). In 17 patients (11.49%), recurrences were observed (13 local recurrences, 3 lung metastasis, and 1 local recurrence with lung metastasis). Fibrosarcomatous DFSP recurred more frequently than classic DFSP (50% vs. 6.82%, respectively), and its disease-free survival was significantly lower ( p < 0.001). In multivariate Cox regression, the presence of fibrosarcomatous areas was the only factor influencing disease-free survival.

          Conclusions

          It is important to identify the fibrosarcomatous variant since it recurs more frequently and has lower recurrence-free survival. Distant metastases, mainly in the lung, are also more frequent in fibrosarcomatous DFSP.

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          Most cited references25

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          Dermatofibrosarcoma protuberans: A clinicopathologic analysis of patients treated and followed at a single institution.

          Helen J Lewis, C Antonescu, Tracey J. Woodruff … (2000)
          Despite optimal surgical therapy for patients with dermatofibrosarcoma protuberans (DFSP), some patients still continue to develop local recurrence. The authors' objective was to identify and analyze clinicopathologic factors for disease free survival in a large group of patients who were followed prospectively at a single institution. Prospectively collected data and pathology slides were available for review from 159 patients with primary or recurrent DFSP who underwent treatment between July 1950 and July 1998. The study group was comprised of patients with either the "classic" form of DFSP or the fibrosarcomatous "high grade" variant of DFSP (FS-DFSP). Patient, tumor, pathologic, and treatment factors were analyzed using the log rank test for univariate influence and Cox regression analysis for multivariate influence. Local recurrence free survival was determined by the Kaplan-Meier actuarial method. Of the 159 patients who comprised the current study group, 134 (84%) had the classic form of DFSP. The FS-DFSP variant was found in the remaining 25 patients (16%). The overall 5-year local recurrence free survival rate was 75%, with a median follow-up of 4. 75 years. The 5-year recurrence free survival rate for each group was 81% and 28%, respectively. On univariate analysis, age > 50 years, very close (< 1 mm) to positive microscopic margins, FS-DFSP variant, high mitotic rate, and increased cellularity were unfavorable prognostic factors. Multivariate analysis determined very close (< 1 mm) to positive microscopic margins and FS-DFSP variant to be independent adverse prognostic factors. For the 34 patients who developed a recurrence after surgical resection (21%), the median time to local recurrence was 32 months. Of the patients in this group, two died from metastatic disease. The prognosis after surgical resection with negative and sometimes positive microscopic margins for patients with DFSP is very good. However, increased age, high mitotic index, and increased cellularity are predictors of poor clinical outcome. The FS-DFSP variant represents a much more aggressive tumor with metastatic potential. Patients who are treated with curative intent for FS-DFSP should undergo aggressive attempts at complete surgical resection. Patients with recurrent classic DFSP without evidence of adverse prognostic features may benefit from conservative management, especially in the setting of potentially unresectable disease. Copyright 2000 American Cancer Society.
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            Dermatofibrosarcoma protuberans: a comprehensive review and update on diagnosis and management.

            Beatriz Llombart, J Guerrero, Carlos Guillén … (2013)
            Dermatofibrosarcoma protuberans (DFSP) is a rare superficial tumor characterized by high rates of local recurrence and low risk of metastasis. DFSP occurs most commonly on the trunk and proximal extremities, affects all races, and often develops between the second and fifth decade of life. The tumor grows slowly, typically over years. Histologically, several variants of DFSP have been described and should be well characterized to avoid misdiagnosis with other tumors. These include pigmented (Bednar tumor), myxoid, myoid, granular cell, sclerotic, atrophic DFSP, giant cell fibroblastoma, and DFSP with fibrosarcomatous areas. Of all these variants, only the DFSP with fibrosarcomatous areas is high grade, with a higher rate of local recurrence and distant metastasis. DFSP is genetically characterized by the t(17;22)(q22;q13), resulting in the fusion of alpha chain type 1 of collagen gene and platelet-derived growth factor beta gene. This translocation is present in 90% of DFSP and represents a very useful tool in the differential diagnosis of DFSP with other tumors with similar histology. The standard treatment is wide local excision with at least a 2-cm margin. However, local recurrence after apparently adequate surgical excision is well recognized. Mohs micrographic surgery would be the treatment of choice with a better cure rate and maximal conservation of tissue. When surgery is insufficient, clinical evidence has suggested that imatinib mesylate is a safe and effective treatment in DFSP, especially in cases of local advanced or metastatic disease. This article presents an overview of the state of the art in the clinicopathological management of this disease. Copyright © 2013 Elsevier Inc. All rights reserved.
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              Incidence and Survival of Primary Dermatofibrosarcoma Protuberans in the United States

              Kathryn L Kreicher, David E Kurlander, Haley R. Gittleman … (2016)
              Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous sarcoma for which data on risk factors, incidence, and survival are limited.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Dermatology
                Journal ID (iso-abbrev): Dermatology
                Journal ID (hwp): DRM
                Journal ID (publisher-id): DRM
                Title: Dermatology (Basel, Switzerland)
                Publisher: S. Karger AG (Basel, Switzerland )
                ISSN (Print): 1018-8665
                ISSN (Electronic): 1421-9832
                Publication date (Electronic): 16 January 2024
                Publication date (Print): June 2024
                Volume: 240
                Issue: 3
                Pages: 487-493
                Affiliations
                [a ]Department of Dermatology, Hospital Universitari de Bellvitge, IDIBELL, Universitat de Barcelona, Barcelona, Spain
                [b ]Department of Plastic Surgery, Hospital Universitari de Bellvitge, IDIBELL, Universitat de Barcelona, Barcelona, Spain
                [c ]Department of Pathology, Hospital Universitari de Bellvitge, IDIBELL, Universitat de Barcelona, Barcelona, Spain
                Author notes
                Correspondence to: Joaquim Marcoval, jmarcoval@ 123456bellvitgehospital.cat
                Article
                Publisher ID: 536172
                DOI: 10.1159/000536172
                PMC ID: 11168446
                PubMed ID: 38228098
                SO-VID: bc62168e-09ea-4ee1-8d00-50f1b1312f5b
                Copyright © © 2024 The Author(s). Published by S. Karger AG, Basel
                License:

                This article is licensed under the Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC) ( http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission.

                History
                Date received : 19 October 2022
                Date accepted : 2 January 2024
                Date: 2024
                Page count
                Figures: 1, Tables: 3, References: 24, Pages: 7
                Funding
                The authors have no funding sources for this work.
                Categories
                Subject: Brief Report

                ScienceOpen disciplines: Dermatology
                Keywords: dermatofibrosarcoma protuberans,fibrosarcoma-like areas,skin
                Data availability:
                ScienceOpen disciplines: Dermatology
                Keywords: dermatofibrosarcoma protuberans, fibrosarcoma-like areas, skin

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