Dermatofibrosarcoma Protuberans: A Clinicopathologic and Therapeutic Analysis of 254 Cases at a Single Institution.
Adolescent
Adult
Aged
Chemotherapy, Adjuvant
/ methods
Child
Dermatofibrosarcoma
/ diagnosis
Dermatologic Surgical Procedures
/ methods
Disease-Free Survival
Female
Follow-Up Studies
Humans
Incidence
Kaplan-Meier Estimate
Male
Margins of Excision
Middle Aged
Neoplasm Recurrence, Local
/ epidemiology
Prognosis
Retrospective Studies
Skin
/ pathology
Skin Neoplasms
/ diagnosis
Survival Rate
Young Adult
Journal
Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.]
ISSN: 1524-4725
Titre abrégé: Dermatol Surg
Pays: United States
ID NLM: 9504371
Informations de publication
Date de publication:
01 02 2021
01 02 2021
Historique:
pubmed:
10
8
2020
medline:
27
4
2021
entrez:
10
8
2020
Statut:
ppublish
Résumé
Dermatofibrosarcoma protuberans (DFSP) is a rare low-grade tumor that typically does not metastasize but often recurs. Fibrosarcomatous DFSP (FS-DFSP) is associated with a substantially higher rate of metastasis and a poorer prognosis. This study sought to investigate the epidemiological, histopathological, and clinical characteristics of DFSP, especially with a particular focus on FS-DFSP. Clinical data from 254 patients treated between January 1999 and July 2018 were retrospectively reviewed. Endpoints of the study were the incidence of significant disease-related clinical events. Follow-up data from 211 patients were available for analysis, with a median follow-up time of 38 months (range: 1-196 months). The 5-year recurrence-free survival rate of patients underwent wide-local excision (WLE) was 97.1%. Patients underwent WLE exhibited a significantly decreased recurrence rate relative to patients treated through local excision (2.9% vs 37.7%; p < .001). Fibrosarcomatous DFSP had significantly higher rates of distant metastasis (66.7% [n = 4] vs 2.0% [n = 4]; p < .001) and long-term mortality (50.0% [n = 3] vs 1.5% [n = 3]; p < .001), compared with classical DFSP (C-DFSP). Wide-local excision is an effective means of reducing DFSP recurrence. Rates of metastasis are higher for FS-DFSP than for C-DFSP, with the former having significantly poorer outcomes.
Sections du résumé
BACKGROUND
Dermatofibrosarcoma protuberans (DFSP) is a rare low-grade tumor that typically does not metastasize but often recurs. Fibrosarcomatous DFSP (FS-DFSP) is associated with a substantially higher rate of metastasis and a poorer prognosis.
OBJECTIVE
This study sought to investigate the epidemiological, histopathological, and clinical characteristics of DFSP, especially with a particular focus on FS-DFSP.
MATERIALS AND METHODS
Clinical data from 254 patients treated between January 1999 and July 2018 were retrospectively reviewed. Endpoints of the study were the incidence of significant disease-related clinical events.
RESULTS
Follow-up data from 211 patients were available for analysis, with a median follow-up time of 38 months (range: 1-196 months). The 5-year recurrence-free survival rate of patients underwent wide-local excision (WLE) was 97.1%. Patients underwent WLE exhibited a significantly decreased recurrence rate relative to patients treated through local excision (2.9% vs 37.7%; p < .001). Fibrosarcomatous DFSP had significantly higher rates of distant metastasis (66.7% [n = 4] vs 2.0% [n = 4]; p < .001) and long-term mortality (50.0% [n = 3] vs 1.5% [n = 3]; p < .001), compared with classical DFSP (C-DFSP).
CONCLUSION
Wide-local excision is an effective means of reducing DFSP recurrence. Rates of metastasis are higher for FS-DFSP than for C-DFSP, with the former having significantly poorer outcomes.
Identifiants
pubmed: 32769521
pii: 00042728-202102000-00011
doi: 10.1097/DSS.0000000000002578
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
e26-e30Informations de copyright
Copyright © 2021 by the American Society for Dermatologic Surgery, Inc. Published by Wolters Kluwer Health, Inc. All rights reserved.
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