Epidemiology of Dermatofibrosarcoma Protuberans in Alberta, Canada, from 1988 to 2007

Kuzel, Paul; Metelitsa, Andrei I.; Dover, Douglas C.; Salopek, Thomas G.

doi:10.1111/j.1524-4725.2012.02482.x

Cited by 25 publications

(27 citation statements)

References 24 publications

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“…The few published population-based studies have shown that DFSP is a relatively rare tumour with age-adjusted rates of less than 1 per 100,000 inhabitants per year) [5,6]. Recent increase in incidence may be explained by a wider knowledge of the tumour among pathologists [6].…”

Section: Definition; Pathophysiology; Epidemiologymentioning

confidence: 99%

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Diagnosis and treatment of dermatofibrosarcoma protuberans. European consensus-based interdisciplinary guideline

Saïag

Grob²,

Lebbe

et al. 2015

European Journal of Cancer

131

140

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Section: Definition; Pathophysiology; Epidemiologymentioning

confidence: 99%

“…Recent increase in incidence may be explained by a wider knowledge of the tumour among pathologists [6]. Because of the decline in developed countries of the incidence of HIV-associated Kaposi's sarcoma, DFSP is nowadays in some countries the most common form of skin sarcoma.…”

Section: Definition; Pathophysiology; Epidemiologymentioning

confidence: 99%

Diagnosis and treatment of dermatofibrosarcoma protuberans. European consensus-based interdisciplinary guideline

Saïag

Grob²,

Lebbe

et al. 2015

European Journal of Cancer

131

140

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“…Dermatofibrosarcoma protuberans (DFSP) is a superficial soft tissue sarcoma with an incidence of 4 in 1,000,000. 1,2 DFSP often presents as a slow-growing, nodular, dermal lesion, with unexpectedly wide, impalpable, subcutaneous tumor extensions. It is known for its locally aggressive growth pattern and high local recurrence rates after incomplete resection (range, 2%-21%), but it has a low potential to metastasize (range, 1%-4%).…”

Section: Introductionmentioning

confidence: 99%

“…3 DFSP is most frequently identified in men and women between ages 20 and 40 years. 1,2 The mainstay of treatment is wide local excision, preferably with a surgical margin of at least 3 cm. [4][5][6][7] In 3% to 20% of patients with DFSP, fibrosarcomatous (FS) change is observed on histopathology.…”

Section: Introductionmentioning

confidence: 99%

Outcome after surgical treatment of dermatofibrosarcoma protuberans: Is clinical follow‐up always indicated?

Veld

Coevorden

Grünhagen

et al. 2019

Cancer

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BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma for which clinical examination up to 10 years is recommended. The objective of this study was to identify prognostic factors for recurrences and metastases that can be used to evaluate the validity of follow-up schedules after treatment for DFSP. METHODS: Patients with DFSP who received treatment between 1991 and 2016 at 3 tertiary centers were included. Cox regression analyses were conducted to identify variables associated with the primary endpoints. RESULTS: In total 357 patients were included, with a median age of 38 years (age range, 2-87 years) and a median follow-up of 60 months (interquartile range, 24-115 months). Eighty-one patients developed recurrent disease (22.7%), and the median time to recurrence was 55.5 months (interquartile range, 20-90 months). Of these, 50 tumors (61.7%) were identified by patient self-examination, whereas 3 recurrences (3.7%) were identified at clinical surveillance. For the remaining 28 tumors, no information was available on how the recurrences were identified (34.6%). Fibrosarcomatous change (hazard ratio, 21.865; P < .001), and positive resection margins (hazard ratio, 14.645; P < .001), were independent prognostic factors for recurrence. Metastases occurred in 4 patients (1.1%). All tumors were identified by imaging after patients presented with symptomatic metastases. Fibrosarcomatous change (P < .001) and tumor size >5 cm (P = .014) were associated with the development of metastases. CONCLUSIONS: Disease recurrence after resection of DFSP remains a significant issue, whereas metastases are uncommon. The majority of recurrences are identified by patient self-examination. Consideration should be given to individualized follow-up schedules based on risk factors for recurrences and metastases. Cancer 2019;125:735-741.

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“…The proportion of published pediatric cases ranges from 4.5% to 20% (3,(9)(10)(11)(12). Although there is unanimous agreement that DFSP is uncommon in children, it has been the subject of little epidemiologic research in this age group (13)(14)(15)(16)(17)(18)(19). Fewer than 200 cases have been reported in the literature (8,20,21).…”

mentioning

confidence: 99%

Pediatric Dermatofibrosarcoma Protuberans in Madrid, Spain: Multi‐institutional Outcomes

Valdivielso-Ramos

Torrelo

Campos‐Domínguez

et al. 2014

Pediatric Dermatology

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Little is known about the incidence and management of dermatofibrosarcoma protuberans (DFSP) in children. We conducted a retrospective review of all patients younger than 18 years of age treated for DFSP over a period of 11 years (2000-2011) in Madrid, Spain. The sample consisted of 13 children. The average annual incidence of DFSP in the pediatric population corresponded to 1.02 cases per million person-years (95% confidence interval 0.55, 1.73). Sites of involvement were diverse, with 15.3% of tumors found in acral locations. The median tumor size was 3.5 cm × 3 cm and the median time from apparent onset to diagnosis was 36 months. Histopathologic examination revealed conventional (77.0%), pigmented (15.4%), and myxoid (7.6%) variants. The mitotic index was consistently <5 per 10 high-power fields. All lesions were removed using surgical excision. One patient developed a local recurrence because of initial affected margins; none developed metastases. The median duration of clinical follow-up was 70.5 months. This study estimated the average annual incidence rate of DFSP in a population of patients younger than 18 years and reviewed the experience of several hospitals in the management of this tumor.

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Epidemiology of Dermatofibrosarcoma Protuberans in Alberta, Canada, from 1988 to 2007

Abstract: The age-standardized incidence of DFSP observed is nearly twice as high as previously reported and has remained stable. The incidence is increasing in women and decreasing in men. DFSP primarily affects young to middle-aged adults and most commonly presents on the trunk.

Cited by 25 publications

References 24 publications

Diagnosis and treatment of dermatofibrosarcoma protuberans. European consensus-based interdisciplinary guideline

Diagnosis and treatment of dermatofibrosarcoma protuberans. European consensus-based interdisciplinary guideline

Outcome after surgical treatment of dermatofibrosarcoma protuberans: Is clinical follow‐up always indicated?

Pediatric Dermatofibrosarcoma Protuberans in Madrid, Spain: Multi‐institutional Outcomes

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