Prognostic impact of fibrosarcomatous transformation in dermatofibrosarcoma protuberans: A cohort study

Background Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade cutaneous malignancy that sometimes transforms into a high-grade fibrosarcomatous variant (DFSP-FS). Limited data compare clinical features and biological behavior of these 2 entities. Objective We sought to compare clinical feat...

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Bibliographic Details
Published in:Journal of the American Academy of Dermatology 2015-03, Vol.72 (3), p.419-425
Main Authors: Hoesly, Paul M., MD, Lowe, Garrett C., MD, Lohse, Christine M., MS, Brewer, Jerry D., MD, Lehman, Julia S., MD
Format: Article
Language:English
Subjects:
Adolescent
Adult
Aged
Aged, 80 and over
Cell Transformation, Neoplastic
Child
Child, Preschool
Cohort Studies
cutaneous signs of disease
Dermatofibrosarcoma - pathology
dermatofibrosarcoma protuberans
Dermatology
Female
fibrosarcoma
Humans
Male
Middle Aged
oncogenesis
pathology
Prognosis
Retrospective Studies
Skin Neoplasms - pathology
surgery
Young Adult
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Summary:Background Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade cutaneous malignancy that sometimes transforms into a high-grade fibrosarcomatous variant (DFSP-FS). Limited data compare clinical features and biological behavior of these 2 entities. Objective We sought to compare clinical features and biological behavior of DFSP and DFSP-FS. Methods This was a retrospective cohort study of ambulatory patients with DFSP or DFSP-FS treated between January 1955 and March 2012 in the dermatology department of a tertiary care academic medical center. Results Of 188 patients, 171 (91%) had DFSP and 17 (9%) had DFSP-FS. Recurrence-free survival differed significantly between the groups over time ( P  = .002). The 1-year and 5-year recurrence-free survival was 94% and 86%, respectively, for DFSP, vs 86% and 42%, respectively, for DFSP-FS. Metastatic disease occurred in no patients with DFSP and in 18% (3 of 17) with DFSP-FS ( P < .001). There were no statistically significant differences in age at diagnosis, sex, race, symptomatology, maximum tumor size, muscle/bone invasion, or duration of tumor before diagnosis. Limitations The retrospective nature of study was a limitation. Conclusions DFSP-FS exhibits more aggressive behavior than DFSP, with lower recurrence-free survival and greater metastatic potential. Their similar clinical presentation mandates histopathological differentiation for prognosis.
ISSN:0190-9622
1097-6787
DOI:10.1016/j.jaad.2014.11.020