Dermatofibrosarcoma protuberans: a population-based cancer registry descriptive study of 66 consecutive cases diagnosed between 1982 and 2002

Department of Dermatology, Orléans Hospital, Orléans, France.
Journal of the European Academy of Dermatology and Venereology (Impact Factor: 3.11). 12/2006; 20(10):1237-42. DOI: 10.1111/j.1468-3083.2006.01780.x
Source: PubMed

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.

  • Source
    • "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. "
    [Show abstract] [Hide abstract]
    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 · 6.37 Impact Factor
  • Source
    • "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. "
    [Show abstract] [Hide abstract]
    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 · 1.29 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Dermatofibrosarcoma protuberans (DFSP) is a relatively rare neoplasm affecting the skin. It is an infiltrative tumour of intermediate malignancy, with a limited potential for metastasis but a high rate of recurrence. The incidence in children is even less frequent, although a proportion of those identified in adulthood may reflect a delay in diagnosis of childhood DFSP. We report the experience of DFSP seen at The Children's Hospital at Westmead (Sydney, Australia). Three children aged 5, 10 and 11 years of age underwent surgical excision of their lesions. Recurrence was evident in one child whose initial histopathology was not definitive for DFSP, and whose initial surgery had not involved wide local excision. All three children were male, and all had lesions affecting their trunk. One child whose lesion was thought to have been evident since birth may have represented congenital DFSP.
    Journal of Plastic Reconstructive & Aesthetic Surgery 01/2008; 62(6):819-23. DOI:10.1016/j.bjps.2007.11.009 · 1.47 Impact Factor
Show more