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Dermatofibrosarcoma protuberans of the face: A clinicopathologic and molecular study of 34 cases
Qianqian Han1, Min Chen1, Jieliang Yang1
1Department of Pathology, West China Hospital, Sichuan University, Chengdu, Sichuan, China.
Background:
Dermatofibrosarcoma protuberans (DFSP) is one of the most common dermal sarcomas, but facial DFSP is rare.
Patients And Methods:
The clinicopathological characteristics of 34 facial DFSPs were reviewed. Additional immunostaining (CD34) and PDGFB/COL1A1-PDGFB fluorescence in situ hybridization (FISH) detection were performed.
Results:
Patients were aged from 24 to 64 years (mean 42.9 years), with a male-to-female ratio of 4.7 : 1. Morphologically, classic DFSP (25/34, 73.5 %), pigmented DFSP (2/34, 5.9 %), DFSP with myoid differentiation (1/34, 2.9 %) and fibrosarcomatous DFSP (FS-DFSP) (6/34, 17.6 %) were found. Moreover, myxoid degeneration was observed in three FS-DFSP cases (3/6, 50.0 %). All 29 cases that underwent CD34 immunohistochemistry exhibited positive staining (100 %). Genetically, PDGFB rearrangement/COL1A1-PDGFB fusion was detected in 94.1 % (16/17) of patients. Regarding prognosis, the recurrence (83.3 % vs. 59.1 %) and metastasis (33.3 % vs. 0 %) rates were higher in FS-DFSPs than that in ordinary DFSPs. All available surgical margins were positive before DFSPs recurrence. All patients with negative excision or re-excision margins were alive without evidence of disease (mean, 81.8 months; median, 81 months).
Conclusions:
Facial DFSP occurs predominantly in males, while FS-DFSPs are more likely to exhibit myxoid degeneration and a worse prognosis. Notably, negative surgical margin status determined a satisfactory prognosis.
