Dermatofibrosarcoma Protuberans: The experience of Evaggelismos General Hospital
Folinas Konstantinos *1, Vaslamatzis Michael 3, Alevizopoulos Nektarios 3, Tegos Theodoros 3,
Valentina Apostolopoulou 4, Cleopatra Rapti 1, Bαrtzi Dimitra 1, Alexandros Georgiou 2,
Eleni Athanasiou 2, Vourlakou Christine 2
*Correspondence to: Folinas Konstantinos, 251 Airforce General Hospital, Athens,Greece.
Copyright
© 2026 Folinas Konstantinos is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Received: 11 June 2026
Published: 01 July 2026
DOI: https://doi.org/10.5281/zenodo.21070570
Abstract
Dermatofibrosarcoma protuberans (DFSP) is the most common sarcoma of the skin. It arises as a, clinically, slow-growing low-to-intermediate malignant sarcoma, occurs most commonly in middle-aged adults. It is not common for DFSP to metastasize but its nature is to be rather aggressive locally with frequent local recurrence. In certain cases, there are areas of fibrosarcoma that have a negative prognostic value as far as recurrences are concerned. We evaluated the clinical presentation of 14 total cases of histopathologically proven cases of DFSP from September of 2020 until July of 2023.The 14 patients consist of 6 females and 8 males patients (F:M 3:4) with ages spanning from 28 to 92 years of age (mean age of 60,54y).The site of distribution were, in most cases: the lungs (2/14),the scalp (2/14),the back (2/14).The follow-up period was ranging from 8 to 41 months (mean period of time 21 months).Dermatofibrosarcoma resembles with other cutaneous clinical entities and the preoperative histopathological diagnose will ensure an adequate surgical excision with clear margins. According to the latest oncological guidelines, adjuvant imatinib with or without radiotherapy will be the treatment of choice.
Keywords: Dermatofibrosarcoma protuberans, Recurrence, Advanced tumor.
Introduction
Dermatofibrosarcoma protuberans (DFSP) is, clinically, a slow-growing, low- to intermediate-grade malignant sarcoma and frequently occurs in middle-aged adults. DFSP was first described in 1890 by Taylor as a sarcoma resembling a keloid.
The annual incidence is reported to be 0.8-4.5 cases per million in USA, and the incidence among African Americans is almost double. Although it appears mostly in adults (20-50 years)7, various cases series report an incidence of 6-20 % in childhood and can even be congenital. It occurs mostly sporadically in children with adenosine deaminase-deficient severe combined immunodeficiency (ADA-SCID).
It is a rare, low to intermediate grade soft tissue sarcoma deriving from the dermal layer of the skin. Lesions tend to grow slowly and may originally present as a painless, skin-colored plaque with possible dark red or blue discoloration. At latter stages, DFSPs can increase in size and become protuberant or ulcerative.
The most common location of DFSP is the trunk (42- 72 %) followed by proximal extremities (20-30 %), and head and neck (10-16 %)10. DFSP sites include surgical scars, old burns, trauma, radiation dermatitis, vaccination sites, central venous line puncture sites and even insect bites.
Ultra structurally, DFSP is characterized by stellate or spindle cells, with long, slender, ramified cell processes joined by primitive junctions, which are similar to dermal dendrocytes.
Although DFPS has traditionally been classified as a fibrohistiocytic tumor, the histogenesis of DFSP remains uncertain. According to different studies, the origin of DFSP may be fibroblastic, histiocytic, or neural. CD34+ dermal dendrocytes have been proposed as another possible origin.
Cytogenetically, more than 90% of DFSP have at (17; 22) (q22; q13), leading to the formation of COL1A1-PDGFB fusion transcripts. Molecular detection of gene rearrangements or fusion transcripts is beneficial not only for the diagnosis in cases without typical morphology, but also for screening patients who are candidates for using imatinib mesylate (a tyrosine
kinase inhibitor that affects PDGFβR). This provides neoadjuvant targeted therapy for the patients with unresectable, recurrent or metastatic DFSP [3]. The standard treatment of respectable DFSPs is complete surgical excision with either wide local excision with tumor free margins or Mohs micrographic surgery, or, rarely, amputation. Mosh surgery would be especially indicated in cases with fibro sarcomatous areas, although in some cases, it may not be detected until the histopathological study of the completely removed tumour.
Materials and Methods
All cases diagnosed as Dermatofibrosarcoma protuberans (DFSP) from September of 2020 until the July of 2023 in the database of the Pathology Department of Evaggelismos General Hospital, Athens, Greece, were include in this study. The cases were then categorizes according to race, age, sex, time of diagnosis, ,evolution to diagnosis, diameter of the lesion, location, previous scarring, pigmentation (Bednar tumour), fibro sarcomatous conversion, recurrence, whether COL1A1-PDGFB t(17;22)(q21.3;q13.1) was observed,ki-67%,CD34 positivity, mitotic activity and surgical margins.
Results
Fourteen patients have been diagnosed with Dermatofibrosarcoma in our hospital (8 male/6 females).Most patients were Caucasian (twelve) and two of unclear descent. The age of the patients ranged from 28 to 92 years (mean age 60,54y and median age 64y).The evolution of diagnosis ranged from 8 to 41 months (mean time 21,6m,median time 21m) and tumour max diameter ranged from 0.5 to 14 centimeters (mean max diameter 7,2cm,median max diameter 5.5cm).
Tumour were located in the head(2 cases),skin (2 cases with no definite location),back (2 cases),lungs (2 cases),abdominal cavity (1 case), left scapula (1 case),right femur (1 case), neck(1 case) and the left leg (1 case).There was no case of the pigmented variant of DFSP (Bednar tumour) and there was no previous scoring noted on the pathology report.
Fibrosarcoma-like areas were histopathologically identified in six of the fourteen cases. It must be noted that in all cases focal necrosis was observed and the mitotic activity was increased in five cases, mild in four cases and small in one case.Ki-67% ranged from <5% to 65% (10-30% average in all cases).CD34 positivity (immunoreactivity) was observed in half (seven) cases. It must be noted that in all fourteen cases,COL1A1-PDGFB t(17;22)(q21.3;q13.1) was observed.
All fourteen patients underwent wide surgical excision with the respective margins being clear in twelve of them. Two of those patients local tumour recurrence (14,3%) and three of these patients had metastatic disease (in the lungs with two of them had fibrosarcoma-like areas).The median tumor size was 7.2 centimeters (median tumor size 5.5cm) ranging from 0.5cm to 14cm.We did not observe differences in the locations of DFSP lesions and fibromatous DFSP lesions, as observed in other studies also.
Discussions
Dermatofibrosarcoma protuberans (DFSP) consists of 1-8% of all soft tissue sarcomas in adults and 18% of all cutaneous sarcomas in adults. It represents 1% of all malignant neoplasms and there are important racial differences, with the incidence being double in African Americans than Caucasians. It appears in the fourth decade of life, unlike other cutaneous neoplasms. It initially presents as an asymptomatic indurated plaque that slowly grows in months or years. The most frequently reported location of DFSP is the
trunk and the proximal region of the extremities. Metastasis to lymph nodes and internal organs is extremely rare. The lung is the organ most frequently affected by distant metastases, but brain, bone, and soft tissue metastases have also been reported. Fibro sarcomatous DFSP is considered an intermediate grade sarcoma and accounts for 5–20% of all DFSP. It seems obvious that the presence of intermediate grade sarcoma areas within a low-grade lesion may be associated with more aggressive behaviour. However, there are few studies on fibro sarcomatous DFSP, and their results are discordant. Mosh surgery would be especially indicated in cases with fibro sarcomatous areas, although in some cases, it may not be detected until the histopathological study of the completely removed tumour.
Limitations of the present study are that it is a retrospective observational survey with a limited number of patients. In addition, the data were obtained from a single reference hospital, and it is possible that the analysed population included patients who were more likely to have non-re presentive tumours.
References