Dermatofibrosarcoma protuberans with fibrosarcomatous areas. A clinicopathologic study of nine cases and a comparison with allied tumors

Abstract

Nine cases of dermatofibrosarcoma protuberans with fibrosarcomatous areas (DFSP with FS) were analyzed clinicopathologically, and the findings compared with allied tumors comprising 43 cases of ordinary DFSP, ten of superficial fibrosarcoma (SFS), and 11 of storiform variant of malignant fibrous histiocytoma (SV MFH). All but one case of DFSP with FS developed on the trunk of the body. Eight of the nine patients (89%) had one recurrence with a shorter prerecurrent interval than that of the ordinary DFSP, and a hipbone metastasis was identified in one patient. These findings reflect the unfavorable course, in contrast to the ordinary DFSP. Microscopically, the FS areas were presented either in the initial lesion (five cases) or in the recurrent one (four cases), areas showing cellularity, mitotic figures, and cytologic anaplasia that exceeded those in the DFSP areas. Dermatofibrosarcoma protuberans with FS and allied categories such as SFS and SV MFH shared various clinical and histologic features but there was a diversity in the prognosis relative to the grade of malignancy. The relationship between DFSP with FS and the allied tumors was given attention.

Detection of COL1A1-PDGFB fusion transcripts and PDGFB/PDGFRB mRNA expression in dermatofibrosarcoma protuberans

“…17 It has been reported that in cases with a fibrosarcomatous area in ordinary dermatofibrosarcoma protuberans or with fibrosarcoma in the recurrent or metastatic lesion, the tumor shows an aggressive clinical course. [18][19][20][21][22][23] By using a microdissection technique, Wang et al 24 detected identical COL1A1-PDGFB fusion transcripts in the two cellular components of dermatofibrosarcoma protuberans with fibrosarcomatous areas. In the present study, we detected COL1A1-PDGFB fusion transcripts in 42 of 57 cases that included 10 dermatofibrosarcoma protuberans with fibrosarcomatous areas, four SAF, three myxoid dermatofibrosarcoma protuberans, and three Bednar tumor.…”

Low-grade Sarcomas with CD34-Positive Fibroblasts and Low-Grade Myofibroblastic Sarcomas

“…Fibrosarcoma can arise in DFS, both de novo and in recurrent lesions [9,[26][27][28][29][30][31]. It is characterized by greater cellularity with fascicular architecture and increased mitotic activity ( Figure 5).…”

Distant Metastasis of Dermatofibrosarcoma Protuberans of the Scalp-Case Report-

“…DFSP contains fibrosarcomatous areas appearing as a fascicular pattern and usually plumper cells with more atypia as shown in this case. 6,22) Sarcomatous areas have lower immunoreactivity for CD34 than DFSP. 9) Sarcomatous areas are more commonly encountered in recurrent lesions and are associated with more local recurrence.…”