Paul M Hoesly1, Garrett C Lowe2, Christine M Lohse3, Jerry D Brewer2, Julia S Lehman4. 1. Feinberg School of Medicine, Northwestern University, Chicago, Illinois. 2. Department of Dermatology, Mayo Clinic, Rochester, Minnesota. 3. Division of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, Minnesota. 4. Department of Dermatology, Mayo Clinic, Rochester, Minnesota. Electronic address: lehman.julia@mayo.edu.
Abstract
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.
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.
Authors: Ana Collazo Lorduy; Berta Obispo; Karen Villar; David Buchser; Jacobo Rogado; Maria Dolores Fenor de la Maza; Yolanda Lage Alfranca; Ramon Colomer Journal: Rare Tumors Date: 2015-12-29
Authors: Jian-Xia Xiong; Tao Cai; Li Hu; Xiao-Li Chen; Kun Huang; Ai-Jun Chen; Ping Wang Journal: World J Clin Cases Date: 2021-07-16 Impact factor: 1.337