Original article
Dermatofibrosarcoma protuberans: A clinicopathological, immunohistochemical, genetic (COL1A1-PDGFB), and therapeutic study of low-grade versus high-grade (fibrosarcomatous) tumors

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Background

Dermatofibrosarcoma protuberans (DFSP) is an uncommon cutaneous tumor, usually low grade, except for the fibrosarcomatous variant (DFSP-FS).

Objectives

We sought to compare the clinicopathological, immunohistochemical, genetic, and therapeutic features between DFSP and DFSP-FS.

Methods

The clinicopathological features were reviewed in 63 DFSP and 12 DFSP-FS. Immunohistochemistry and multiplex reverse transcriptase-polymerase chain reaction were carried out using formalin-fixed, paraffin-embedded tissue, using specific primers for collagen type I alpha 1 (COL1A1) and platelet-derived growth factor beta (PDGFB).

Results

DFSP-FS was associated with tumor history longer than 5 years (P = .009), tumor size greater than 4 cm (P = .001), more stages of modified Mohs micrographic surgery (P = .005), expansive subcutaneous infiltration (P = .005), muscular invasion (P = .0001), absence of CD34 staining (P = .018), p53 positivity (P = .006), and increased proliferative activity (P = .004) compared with DFSP. The COL1A1-PDGFB fusion transcript was found in 100% DFSP-FS and 72% DFSP. No association was found between the different COL1A1-PDGFB fusion transcripts and the different histologic subtypes. Wide local excision (2 cm) was performed in 47% of cases and modified Mohs micrographic surgery in 53%. After a mean follow-up of 73 months (range 21-235), 6 patients had local recurrence (5 DFSP, 1 DFSP-FS) and one died of disease (DFSP-FS). The only factor related to local recurrence was the type of surgery (17% wide local excision vs 0% modified Mohs micrographic surgery) (P = .006).

Limitations

Our study is retrospective. Prospective studies are necessary to confirm our results.

Conclusions

DFSP-FS reflects tumor progression in DFSP, with larger size, particular invasive patterns, p53 expression, and increased proliferative activity. However, as in low-grade DFSP, appropriate surgery permits a tumor-free excision. COL1A1-PDGFB is a useful tool for diagnosis of DFSP and particularly for DFSP-FS.

Section snippets

Case selectionclinicopathological 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.

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