Pediatric Dermatofibrosarcoma Protuberans: Multi-Institutional Outcomes

Iqbal, Corey W.; Peter, Shawn St.; Ishitani, Michael B.

doi:10.1016/j.jss.2011.01.042

Cited by 28 publications

(31 citation statements)

References 18 publications

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“…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%

“…The duration of clinical follow-up in our study (median 70.5 months [IQR 30.7-123.0 months]) was similar to the follow-up periods of other published series of 5.8 years (14). 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).…”

Section: Discussionsupporting

confidence: 85%

“…Such lesions may be difficult to distinguish clinically from other, more commonly occurring lesion types, such as morphea or vascular lesions, which often causes major delays in diagnosis (8,16,32). Tumor size at the time of diagnosis normally ranges from 1 to 5 cm, and DFSP tends to grow slowly over several years, eventually becoming quite large if left untreated (14,33,34). We found a median size of 3.5 cm 9 3 cm (IQR 1.75 cm 9 5.5 cm-1.15 cm 9 3.5 cm) and a mean size of 4.4 cm 9 2.8 cm AE 3.8 cm 9 2 cm.…”

Section: Discussionmentioning

confidence: 99%

“…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).…”

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confidence: 99%

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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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Section: Discussionsupporting

confidence: 90%

Section: Discussionsupporting

confidence: 85%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

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

Valdivielso-Ramos

Torrelo

Campos‐Domínguez

et al. 2014

Pediatric Dermatology

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“…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.…”

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confidence: 99%

Dermatofibrosarcoma protuberans in a 10-year-old child

Wollina

2013

J Dermatol Case Rep

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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.

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Dermatofibrosarcoma Protuberans (DFSP) and Variants

Sbaraglia¹

2020

Encyclopedia of Pathology

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Pediatric Dermatofibrosarcoma Protuberans: Multi-Institutional Outcomes

Cited by 28 publications

References 18 publications

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

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

Dermatofibrosarcoma protuberans in a 10-year-old child

Dermatofibrosarcoma Protuberans (DFSP) and Variants

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