Dermatofibrosarcoma protuberans: a population-based cancer registry descriptive study of 66 consecutive cases diagnosed between 1982 and 2002.
ABSTRACT Dermatofibrosarcoma protuberans (DFSP) is a rare malignant tumour of the skin, with an estimated incidence of 0.8 to five cases per 1 million people per year.
To study epidemiological, immunohistochemical and clinical features, delay in diagnosis, type of treatment and outcome of DFSP from 1982 to 2002.
Using data from the population-based cancer registry, 66 patients with pathologically proved DFSP were included (fibrosarcomatous DFSP were excluded). Each patient lived in one of the four departments of Franche-Comté (overall population of 1 million people) at the time of diagnosis. The main data sources came from public and private pathology laboratories and medical records. The rules of the International Agency for Research on Cancer were applied.
The estimated incidence of DFSP in Franche-Comté was about three new cases per 1 million people per year. Male patients were affected 1.2 times as often as female patients were. The trunk (45%) followed by the proximal extremities (38%) were the most frequent locations. DFSP occurred mainly in young adults between 20 and 39 years of age. Mean age at diagnosis was 43 years, and the mean delay in diagnosis was 10.08 years. Our 66 patients initially underwent a radical local excision. Among them, 27% experienced one or more local recurrences during 9.6 years of follow-up. There was one regional lymph node recurrence without visceral metastases. These recurrences were significantly related to the initial peripheral resection margins. We observed a local recurrence rate of 47% for margins less than 3 cm, vs. only 7% for margins ranging from 3 to 5 cm [P=0.004; OR=0.229 (95%, CI=0.103-0.510)]. The mean time to a first local recurrence was 2.65 years. Nevertheless, there was no death due to the DFSP course at the end of the follow-up, and the final outcome was favourable.
Our study emphasizes the importance of wide local excision with margins of at least 3 cm in order to prevent local recurrence. However, the recent development of inhibitors of signal transduction by the PDGFB pathway should soon modify the surgical strategy, which is often too mutilating.
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ABSTRACT: To our knowledge, no systematic review of dermatofibrosarcoma protuberans (DFSP) outcomes based on the presence or absence of fibrosarcomatous (FS) change has been performed. We sought to compare available outcome data for DFSP versus DFSP-FS. The literature was searched for DFSP and DFSP-FS reports with outcome data (local recurrence, metastasis, or death from disease). Chi-square tests were calculated to determine whether DFSP and DFSP-FS significantly differed in risk of local recurrence, metastasis, and death from disease. In all, 24 reports containing 1422 patients with DFSP and 225 with DFSP-FS are summarized. Risk of local recurrence, metastasis, and death from disease in DFSP-FS was significantly higher as compared with DFSP (local recurrence 29.8% vs 13.7%, risk ratio 2.2 [95% confidence interval 1.7-2.9]; metastasis 14.4% vs 1.1%, risk ratio 5.5 [95% confidence interval 4.3-7.0]; and death from disease 14.7% vs 0.8%, risk ratio 6.2 [95% confidence interval 5.0-7.8]). There was no significant difference in DFSP-FS outcomes based on proportion of FS change within tumors. This study is based on previously reported data from different hospitals with no uniform process for reporting FS change. The impact of confounders (age, immune status, tumor location, treatment) could not be evaluated because of limited data. Based on available retrospective data, risk of metastasis and death is elevated in DFSP-FS as compared with DFSP. Even a low degree of FS involvement portends worse outcomes.Journal of the American Academy of Dermatology 04/2014; · 4.91 Impact Factor
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ABSTRACT: Abstract Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous sarcoma that frequently recurs locally, but rarely metastasizes. The purpose of this work is to present a clinical series of DFSP patients and national Danish incidence data in the period 2000-2012. Furthermore, the aim is to present guidelines on the management based on a review of the literature. Medical records of 26 consecutively treated patients at the Department of Plastic Surgery in Health Care Region Zealand were reviewed and histological specimens were reassessed. To investigate national Danish incidence in the period 2000-2012, data were extracted from the national pathology registry. Finally, a literature search was performed in Pubmed and Cochrane, and 23 major publications were reviewed. Studies on Mohs Micrographic surgery were excluded. All patients were treated with wide local excision (WLE) with a median margin of 2.8 cem and a median follow-up time of 36 months. We found a local recurrence rate of 4%. Our national incidence data were based on 374 patients. The overall incidence was 0.53 per 100,000 persons. The prevalence of DFSP in the age group 20-50 years was significantly higher than the group below 20 years (p < 0.0001). Surgery is the treatment of choice for primary DFSP, local recurrences, and metastases. If clear margins cannot be obtained by WLE or surgery is not an option because of unacceptable functional or cosmetic outcome, adjuvant radiotherapy or imatinib can be considered. Chemotherapy can be a final option if other treatments fail.Journal of plastic surgery and hand surgery. 07/2013;
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ABSTRACT: Dermatofibrosarcoma protuberans (DFSP) is a potentially malignant dermal mesenchymal tumour with a high risk of local recurrence. DFSP presents a sprawling appearance whose complete excision requires important margins. DFSP was initially resected with a 5cm excision margins, and more recently 3cm then 2cm margins were recommended. Mohs micrographic surgery (MMS) helps reduce these margins thanks to a 3-dimensional excision around the tumour, which is analysed in its entirety. We used the modified MMS called slow-MMS and tried every time it was possible to perform direct closure. Thirty-five patients presenting a DFSP between 2004 and 2013 within the Plastic Surgery unit at Claudius Regaud Institute were included in this retrospective study. The patients were treated with slow-MMS using paraffin-embedded sections. One surgery was necessary for 72% of patients. For 17%, we had to perform a second surgery, and for 11% a third one. Our median clinical excision margins was 17mm (range 9.0:30.0). After a median follow-up of 46 months (range 35.2:60.2), we didn't observe any recurrence. Only one case required a local flap; for the others, the loss of substance was resolved with a direct closure. Slow-MMS enabled a local control of the margins without recurrence at 46 months in our series. Besides, it helps performing smaller margins than wide excision and thus preserving the tissues. In our opinion, this is the treatment of choice regarding DFSP for which tissue sparing is essential. It seems particularly appropriate near functional areas or on the face.Annales de chirurgie plastique et esthetique 01/2014; · 0.33 Impact Factor