Original articleDermatofibrosarcoma protuberans: A clinicopathological, immunohistochemical, genetic (COL1A1-PDGFB), and therapeutic study of low-grade versus high-grade (fibrosarcomatous) tumors
Section snippets
Case selection–clinicopathological data
All cases of DFSP included in the pathological database of two centers, Hospital Clínico Universitario and Instituto Valenciano de Oncología, Valencia, Spain, from 1990 to 2005, were reviewed. Informed consent from patients was obtained in accordance with the ethical committee procedures of the respective institutions.
Hematoxylin and eosin–stained slides (2-16/case), were reviewed by 3 of the authors (B. L., C. M., A. L-B.). The tumors were categorized histologically as conventional DFSP versus
Histopathological findings
In all, 75 cases of DFSP were reviewed. All tumors included areas of conventional DFSP composed of a proliferation of uniform spindle tumor cells with slender nuclei arranged in a storiform or cartwheel pattern, with intercellular collagen deposition and small capillary blood vessels scattered throughout (Fig 1, A). At least focally, the diffuse honeycomb infiltration into underlying fatty tissue was seen (Fig 1, B). The DFSP subtypes are specified in Table I.
In contrast to the usual low-grade
Discussion
DFSP possesses a significant risk of local recurrence, but a limited risk of distant metastasis. Although it seems logical that higher-grade areas within a low-grade lesion would adversely affect behavior, previous studies failed to statistically confirm this hypothesis.27, 28, 29 To our knowledge, our study is unprecedented for DFSP, in that the clinical, pathological, immunohistochemical, and biological characteristics, as well as treatment and outcome of 75 cases of high-grade (DFSP-FS) and
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Supported by Fomación en Investigación en Salud grant P1040822 and grant GV06/274 from the Consellería de Educación y Ciencias (Generalitat Valenciana).
Conflicts of interest: None declared.