Monnier D, Vidal C, Martin L et al.Dermatofibrosarcoma protuberans: a population-based cancer registry descriptive study of 66 consecutive cases diagnosed between 1982 and 2002. J Eur Acad Dermatol Venereol 20:1237-1242

Department of Dermatology, Orléans Hospital, Orléans, France.
Journal of the European Academy of Dermatology and Venereology (Impact Factor: 2.83). 12/2006; 20(10):1237-42. DOI: 10.1111/j.1468-3083.2006.01780.x
Source: PubMed


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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    • "No evidence of hereditary or familial predisposition exists. The 5-year relative survival rates for reported in all population-based studies are can reach up to 100% [7,8,12]. "
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    ABSTRACT: Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous neoplasm associated with a high cure rate. We present a case of aggressive DFSP with fibrosarcomatous areas in the head and neck. A 28-year-old Mediterranean female presented with a 45-day history of rapidly growing cutaneous lesion of the face. Surgical biopsy confirmed the diagnosis of DFSP. Subsequently, the patient underwent wide local surgical resection, followed by reconstruction. Histopathology report revealed fibrosarcomatous transformation and the patient underwent adjuvant radiotherapy. The patient continues to be disease free at the 35-month follow-up. Although DFSP behave as non-aggressive malignancy, surgery with complete removal of the affected area is the intervention of choice. Moreover, adjuvant treatment and follow-up of the patient is essential in order to prevent recurrence.
    Head & Neck Oncology 02/2011; 3(1):5. DOI:10.1186/1758-3284-3-5 · 3.14 Impact Factor
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    • "With population projections predicting a significant increase in the elderly population, skin cancer will continue to be a growing public health problem. Similar to previous reports, DFSP incidence rates were comparable between men and women, and highest for tumors occurring on the trunk (Monnier et al., 2006; Dimitropoulos, 2008; Kampshoff and Cogbill, 2009). However , interestingly, we found that DFSP incidence was decreasing significantly over time for tumors at sun-exposed sites, yet there is no clear explanation for this. "
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    ABSTRACT: Little is known regarding the incidence trends of squamous cell skin carcinoma (SCC) and rare skin tumors, including Merkel cell carcinoma (MCC) and dermatofibrosarcoma protuberans (DFSP). The purpose of this study was to examine recent incidence trends for these cancers from 1990 to 2005 using the population-based Swedish National Cancer Registry. SCC incidence increased significantly over the study period and increased sharply with age. MCC incidence increased significantly for men for tumors at sun-exposed sites, and increased sharply with age. DFSP incidence was similar for sun-exposed and covered sites and decreased significantly only at exposed sites. A combination of behavioral, biological, and environmental factors likely explains the trends observed for these skin cancers.
    Journal of Investigative Dermatology 05/2010; 130(5):1323-8. DOI:10.1038/jid.2009.426 · 7.22 Impact Factor
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    • "Dermatofibrosarcoma protuberans is a rare, painless, monoclonal, cutaneous soft tissue sarcoma of unknown cause that was first described by Taylor in 1890 [5] . It constitutes approximately 1% of all soft tissue sarcomas, shows slight male preponderance and generally presents in the third and fourth decades of life [6] [7] . Its clinical manifestations are non-specific. "
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    ABSTRACT: Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade, cutaneous neoplasm with pronounced tendency for local recurrence. A case of DFSP that showed direct infiltration into the underlying bone marrow is described. To the best of our knowledge, such direct bony involvement by dermatofibrosarcoma has not been reported in the English literature to date. The role of imaging is also discussed for planning adequate initial treatment, which will result in a lower recurrence rate and improved clinical outcome.
    Cancer Imaging 09/2009; 9(1):63-6. DOI:10.1102/1470-7330.2009.0011 · 2.07 Impact Factor
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