Clinicopathologic features and treatment outcomes of dermatofibrosarcoma protuberans: a 25-year retrospective study

Cutaneous DFSP in this series demonstrated clinicopathologic features consistent with those reported in the literature, including a predilection for the trunk and middle-aged females. Histologically, most cases exhibited the classic storiform pattern with strong CD34 positivity. Wide local excision was the primary treatment modality, with a relatively low recurrence rate compared to the literature, possibly influenced by the use of adjuvant radiotherapy in select patients. Although uncommon, metastasis occurred in cases with recurrent disease and fibrosarcomatous transformation. Ongoing research into systemic therapies and molecularly targeted treatments is needed to improve outcomes in advanced DFSP.

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Publication Details

Journal
Anais Brasileiros de Dermatologia
Published
2025-10-27
DOI
https://doi.org/10.1016/j.abd.2025.501213
Primary Topic
Sarcoma Diagnosis and Treatment
Type
article
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article

Clinicopathologic features and treatment outcomes of dermatofibrosarcoma protuberans: a 25-year retrospective study

Lanyu Sun, Diogo de Sousa, Mafalda Pinho, Luís Soares‐de‐Almeida et al.
Anais Brasileiros de Dermatologia
Sarcoma Diagnosis and Treatment
article

Clinicopathologic features and treatment outcomes of dermatofibrosarcoma protuberans: a 25-year retrospective study

Lanyu Sun, Diogo de Sousa, Mafalda Pinho, Luís Soares‐de‐Almeida, Paulo Filipe, Claúdia Brazão, Joana Frade, Pedro Vasconcelos
article en

Abstract

Cutaneous DFSP in this series demonstrated clinicopathologic features consistent with those reported in the literature, including a predilection for the trunk and middle-aged females. Histologically, most cases exhibited the classic storiform pattern with strong CD34 positivity. Wide local excision was the primary treatment modality, with a relatively low recurrence rate compared to the literature, possibly influenced by the use of adjuvant radiotherapy in select patients. Although uncommon, metastasis occurred in cases with recurrent disease and fibrosarcomatous transformation. Ongoing research into systemic therapies and molecularly targeted treatments is needed to improve outcomes in advanced DFSP.

Anais Brasileiros de DermatologiaVol. 100(6)
University of Lisbon (PT), Administração Regional de Saúde de Lisboa e Vale do Tejo (PT), Hospital de Santa Maria (PT)
Openalex Percentile: Top 55%
Sarcoma Diagnosis and Treatment
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Clinicopathologic features and treatment outcomes of dermatofibrosarcoma protuberans: a 25-year retrospective study — Lanyu Sun, Diogo de Sousa, et al. · Anais Brasileiros de Dermatologia (2025) | TGRS Research Map | TGRS