Le dermatofibrosarcome chez l’enfant

Wahab, O. Abdel; Qassemyar, A.; Maillet, M.; Mortier, Laurent; Guerreschi, P.

doi:10.1016/j.anplas.2011.11.002

Cited by 3 publications

(4 citation statements)

References 29 publications

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“…The mean age at diagnosis for cases with acquired lesions was lower than previously reported in other similar series (11.9–13 years). 16,17 Delay of diagnosis was similar to that reported by other authors, which varied from months to 16 years. 9,11,19,24 An indolent course and painful lesions were reported in case series.…”

Section: Discussionsupporting

confidence: 88%

“…The most common location of DFSP lesions in our cases was the trunk followed by the extremities, as previously reported in pediatric series 1,11,16,17,22 ; however, it is important to mention that any anatomic distribution is possible. 9 Congenital DFSP is frequently found in the trunk and upper extremities; however, there are reports of variable anatomic distribution.…”

Section: Discussionsupporting

confidence: 71%

“…There were two similar series from France and the United States, each reporting 15 pediatric acquired cases (including 2 patients at 18 years of age) over a period of evaluation of 13 and 30 years, respectively. 16,17 An additional publication included a group of 28 pediatric patients with DFSP, giant cell fibroblastoma, and hybrid lesions from six institutions and personal consultations over 12 years. 18…”

Section: Discussionmentioning

confidence: 99%

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Dermatofibrosarcoma Protuberans in Pediatric Patients

Rios

Lara‐Corrales

2014

J Cutan Med Surg

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Background: Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue tumor. In children, there are acquired and congenital presentations. Due to clinical similarities with other conditions, diagnosis may be delayed.Objective: To review the clinical characteristics and treatment of DFSP in pediatric patients. Methods: A retrospective chart review was performed from January 2002 to September 2012. Inclusion criteria were patients under 18 years of age with a histopathologic diagnosis of DFSP. Data on demographics, clinical characteristics, treatments, and outcomes were collected.Results: Information was gathered from 17 patients; 9 (53%) were female. Congenital lesions were reported in 7 patients. The mean delay of diagnosis was 5.7 years; the most common anatomic location was the trunk in 8 of 17 (47%) cases. Treatment options included wide local surgery, Mohs surgery, and imatinib mesylate. Conclusion: A detailed medical history and identification of the natural course of common conditions seen in pediatric patients are important to identify less common lesions and to suspect DFSP. Contexte: Le dermatofibrosarcome (DFS) de Darier et Ferrand est une tumeur peu fré quente des tissus mous. Il existe des formes acquises et des formes congé nitales chez les enfants. La pose du diagnostic peut ê tre retardé e en raison de ressemblances cliniques avec d'autres affections. Objectif: L'é tude visait à passer en revue les caracté ristiques cliniques du DFS et de son traitement chez les enfants. Mé thode: Nous avons procé dé à un examen ré trospectif de dossiers mé dicaux, couvrant la pé riode de janvier 2002 à septembre 2012. Les critè res de sé lection comprenaient un â ge infé rieur à 18 ans et un diagnostic histopathologique de DFS. Ont é galement é té recueillies des donné es dé mographiques ainsi que des donné es sur les caracté ristiques cliniques, les traitements, et les ré sultats cliniques.Ré sultats: Les renseignements recueillis provenaient de 17 patients, dont 9 (53%) é taient de sexe fé minin. Des lé sions congé nitales ont é té relevé es chez 7 patients. Le temps moyen é coulé avant la pose du diagnostic é tait de 5.7 ans, et le siè ge le plus fré quent des lé sions é tait le tronc, dans 8 cas sur 17 (47%). Les traitements comprenaient la chirurgie locale é largie, la chirurgie de Mohs, et le traitement par le mé sylate d'imatinib.Conclusion: Une anamnè se dé taillé e et la reconnaissance de l'é volution naturelle d'affections fré quentes observé es chez les enfants sont des é lé ments importants de l'identification de lé sions moins fré quentes et de la pré sence pré sumé e du DFS.

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Section: Discussionsupporting

confidence: 88%

Section: Discussionsupporting

confidence: 71%

Section: Discussionmentioning

confidence: 99%

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Dermatofibrosarcoma Protuberans in Pediatric Patients

Rios

Lara‐Corrales

2014

J Cutan Med Surg

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“…Thirty-two out of 44 studies (n=1824) reported symptoms in all locations 8 23–53. Further analysis restricted to these studies showed similar results.…”

Section: Resultsmentioning

confidence: 68%

Clinical presentation of childhood soft tissue sarcomas: a systematic review and meta-analysis

Ni Cheallaigh,

Liu,

Fern

et al. 2023

Arch Dis Child

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BackgroundTime to diagnosis (TTD) of childhood soft tissue sarcoma (STS) is significantly associated with survival. This review aims to identify pre-diagnostic symptoms/signs to inform earlier diagnosis interventions.MethodsMedline, Embase, Cochrane and Web-of-Science were searched between January 2010 and February 2021 for studies including children (<18 years) diagnosed with STS, with no language restrictions. Pooled proportions of symptoms/signs were calculated and subanalysed by tumour location and age.ResultsFifty-nine eligible studies were identified, totalling 2462 cases. The most frequent symptoms were lump/swelling (38%, 95% CI 27% to 51%), pain (6%, 95% CI 3% to 10%), cutaneous changes (4%, 95% CI 0 to 9%), localised eye swelling (3%, 95% CI 0 to 7%), cranial nerve deficits (2%, 95% CI 0 to 5%) and constitutional symptoms (2%, 95% CI 0 to 5%).Symptoms varied by location and age. Localised eye swelling (20%, 95% CI 3% to 45%), cranial nerve deficits (14%, 95% CI 4% to 28%) and impaired visual function (6%, 95% CI 0 to 17%) were frequent in head and neck tumours. For abdomen/pelvic tumours, urinary symptoms (24%, 95% CI 5% to 15%), abdominal distension/discomfort (22%, 95% CI 4% to 47%), genital lump/swelling (16%, 95% CI 1% to 42%), constitutional symptoms (9%, 95% CI 0%] to 23%), vaginal bleeding (7%, 95%C I 0 to 21%) and bowel habit changes (6%, 95% CI 0 to 17%) were frequent.In <5 years, consumptive coagulopathy (16%, 95% CI 0 to 48%), cutaneous changes (5%, 95% CI 0 to 40%), genital lump/swelling (4%, 95% CI 0 to 14%), reduced mobility (3%, 95% CI 0 to 11%), vaginal bleeding (2%, 95% CI 0 to 11%) and bleeding/bruising/petechiae (2%, 95% CI 0 to 20%) were frequent compared with lump/swelling, constitutional symptoms, pain and headaches which were frequent among >11 years.ConclusionsFor STS, pre-diagnostic symptoms differ by age and location, highlighting the need to tailor early diagnosis interventions.

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