Dermatofibrosarcoma protuberans in children

Reddy, Chaitan; Hayward, Peter G.; Thompson, P. G.; Kan, Alex

doi:10.1016/j.bjps.2007.11.009

Cited by 42 publications

(23 citation statements)

References 36 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In our series, the median age at diagnosis was 15 (IQR 8.5-16), similar to the results of other pediatric series, with a median age at diagnosis of 7 to 16 years (18,19,22). A large series found slight differences in incidence between men and women (2,14,17,21). Girls and boys were affected almost equally in our study.…”

Section: Discussionsupporting

confidence: 90%

“…Recurrence rates have been reported to be in the range of 20% to 50%, with the majority of recurrences occurring within the first 3 years after excision (3,16,21), although late relapses may occur (33). The adequacy of surgical excision remains central to minimizing disease recurrence.…”

Section: Discussionmentioning

confidence: 97%

“…The mitotic index was <5 per 10 high-power fields in the majority of patients. DFSP normally has a low mitotic index and no atypical forms (21). Several variants have been described, including pigmented (Bednar tumor), myxoid, granular cell, atrophic, sclerosing, with fibrosarcomatous areas, and tumors with areas of giant cell fibroblastoma (35,36).…”

Section: Discussionmentioning

confidence: 99%

“…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: 91%

See 3 more Smart Citations

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

Valdivielso-Ramos

Torrelo

Campos‐Domínguez

et al. 2014

Pediatric Dermatology

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionsupporting

confidence: 90%

Section: Discussionmentioning

confidence: 97%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 91%

See 2 more Smart Citations

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

Valdivielso-Ramos

Torrelo

Campos‐Domínguez

et al. 2014

Pediatric Dermatology

View full text Add to dashboard Cite

show abstract

“…Micrographic control of excision margins improves outcome and reduces recurrence rates while limiting the need of closure by flaps or grafts. 11,[24][25][26] We treated our patient with micrographic controlled surgery (delayed MMS). A regular follow-up is necessary to control the outcome and minimize delay for re-treatment when needed.…”

mentioning

confidence: 99%

Dermatofibrosarcoma protuberans in a 10-year-old child

Wollina

2013

J Dermatol Case Rep

View full text Add to dashboard Cite

Background: Dermatofibrosarcoma protuberans is a rare mesenchymal malignancy in childhood and adolescence. The tumor is characterized by dermal spindle cell proliferation with infiltration of subcutaneous tissue, expression of CD34, and a specific fusion of the platelet-derived growth factor beta with the collagen type 1alpha1 gene. Main observation:We observed a 10-year-old girl with a medaillon-like, asymptomatic plaque on the chest that was diagnosed as DSFP. The tumor was completely removed by delayed Mohs surgery. Follow-up so far has shown a complete response. Conclusion:The prognosis of dermatofibrosarcoma protuberans in children is excellent as long as early diagnosis is followed by complete excision with Mohs surgery as a golden standard. (J Dermatol Case Rep.

show abstract