Risk factors for distant metastasis of dermatofibrosarcoma protuberans

Abstract

BackgroundDermatofibrosarcoma protuberans (DFSP) may recur locally but rarely metastasizes. Fibrosarcomatous transformation in dermatofibrosarcoma protuberans (FS-DFSP) is said to have worse prognosis compared with ordinary DFSP (O-DFSP). Since DFSP rarely metastasizes, there have been few reports summarizing data on distant metastasis cases at single institution. The aim of this retrospective study is to review DFSP cases in order to analyze risk factors for metastasis.Patients and methodsThis retrospective study involved 67 patients. We analyzed O-DFSP and FS-DFSP metastasis rates, metastasis sites, time to metastasis, the relationship between frequency of local recurrence and metastasis, and the relationship between primary tumor size and metastasis.ResultsDistant metastasis was found in 5 (7.4 %) of 67 cases with DFSP. Of the five cases, the histopathological diagnosis was FS-DFSP in four cases and O-DFSP in one case. Out of five cases with metastasis, three had not recurred and two had recurred twice. No clear correlation was identified (Fisher’s exact test: p = 0.216). The primary tumor diameters in the metastatic cases were 15.0, 12.6, 20.5, 13.0, and 5.0 cm, respectively. The tumor diameters in metastatic cases were significantly larger (Fisher’s exact test: p < 0.0001).ConclusionsIn this study, we identified a stronger correlation between DFSP metastasis and tumor size. There was a high possibility that the cases with large tumors might be FS-DFSP, having high rate of metastasis and poor prognosis. In treatment of DFSP, early diagnosis before primary tumor growth and wide resection is considered important.Level of evidence V.

Clinical Features, Pathological Findings and Treatment of Recurrent Dermatofibrosarcoma Protuberans

“…In our study, the majority of recurrent lesions also grew slowly at the beginning but on subsequent grew rapidly. K et al reported that patients with large lesions have a high possibility to be associated with FS-DFSP 13. Interestingly, in the present study, there was no significant difference in tumor size between recurrent FS-DFSP and recurrent O-DFSP.…”

Outcome after surgical treatment of dermatofibrosarcoma protuberans: Is clinical follow‐up always indicated?

“…9,10,24 Criscito et al reported that survival was influenced by tumor size, but they did not analyze the relation between recurrence and size. 10,15,26 No metastases were identified in patients without FS changes in this series. 10,24 In the current study, only 4 patients (1.1%) developed distant metastases, indicating that larger DFSPs and those with FS changes are more likely to develop metastases versus smaller DFSPs and those without FS changes.…”

“…Other studies did not report a significant influence of tumor size on recurrences. 3,26 Metastases most often are located in the lung but, in this cohort, also were observed in other locations, such as the pancreas, liver, and bone. The risk of metastases in patients who have DFSP with FS changes has been described previously, but series on this subject are scarce and consist of limited numbers of patients or case reports.…”

“…3,22,23 Only Fields and colleagues did not identify FS change as a factor that influenced recurrence. 3,10,15,25,26 Two reviews reported metastases rates of 13.4% and 14.4% in patients with FS changes and 1.1% in patients without FS changes. The prognostic value of tumor size also is debated in other reports.…”

“…10,24 In the current study, only 4 patients (1.1%) developed distant metastases, indicating that larger DFSPs and those with FS changes are more likely to develop metastases versus smaller DFSPs and those without FS changes. 3,10,25,26 A review by Liang et al indicated that metastases are located mostly in the lungs but also in bone, soft tissue, liver, kidney, the gastrointestinal system, and lymph nodes. 3,10,15,25,26 Two reviews reported metastases rates of 13.4% and 14.4% in patients with FS changes and 1.1% in patients without FS changes.…”