The Prognostic Significance of Fibrosarcomatous Transformation in Dermatofibrosarcoma Protuberans
- 1 April 2006
- journal article
- research article
- Published by Ovid Technologies (Wolters Kluwer Health) in The American Journal of Surgical Pathology
- Vol. 30 (4), 436-443
- https://doi.org/10.1097/00000478-200604000-00002
Abstract
Dermatofibrosarcoma protuberans (DFSP) is a superficial tumor characterized by high rates of local recurrence and a small risk of metastasis. Fibrosarcomatous (FS) areas rarely arise in DFSP, and considerable controversy exists as to whether these tumors have a higher risk of metastasis than the typical DFSP. The aim of this study was to reappraise the prognostic significance of FS changes in DFSP by analyzing 41 patients from the consultation files of our institution. The study included 23 females and 18 males, with a median age of 48 years (range, 16–100 years). Eighteen lesions were located on the trunk, 16 on the extremities, and 7 on the head/neck region. All tumors were treated with local excision, and the surgical margins were considered positive for tumor in 22 of 39 cases (56%). Fibrosarcomas arose de novo in 38 cases and as a recurrence in 3 cases. All tumors involved the dermis and subcutis, and the FS component comprised 5% to 95% of the tumor area (median, 60%). Mitotic rates of the FS component (median, 20 mitoses/10 high-power fields [HPFs]; range, 5–48/10 HPFs) were considerably higher than those of the neighboring DFSP component (0–2 mitoses/10 HPFs). Immunohistochemical analyses showed that CD34 expression was stronger and more extensive in the DFSP component (97% positive; median intensity, 3+) than in the FS component (81% positive; median intensity, 2+). The MIB-1 labeling index was higher in the FS areas (median, 20%; range, 5%–45%) than in the DFSP areas (<3%). Expression of p53 was present in 92% of the FS areas and in only 3% of adjacent DFSP areas. Follow-up data revealed that 8 patients had local recurrences, 4 patients (10%) had metastases, and 2 patients died of disease. None of the variables evaluated, including margin status, FS proportion, and mitotic count, correlated with disease progression. We demonstrate that FS change in DFSP is a form of tumor progression that carries an increased risk of metastasis over classic DFSP and is associated with gains of p53 mutations and increased proliferative activity.Keywords
This publication has 28 references indexed in Scilit:
- Low recurrence rate after surgery for dermatofibrosarcoma protuberansCancer, 2004
- The pathogenesis of cancer metastasis: the 'seed and soil' hypothesis revisitedNature Reviews Cancer, 2003
- Dermatofibrosarcoma protuberansCancer, 2000
- Dermatofibrosarcoma Protuberans with Fibrosarcomatous Areas: A Clinico-pathologic and Immunohistochemic Study in Four CasesThe American Journal of Dermatopathology, 1997
- Dermatofibrosarcoma protuberansJournal of the American Academy of Dermatology, 1996
- Myoid differentiation in dermatofibrosarcoma protuberans and its fibrosarcomatous variant: clinicopathologic analysis of 5 casesJournal of Cutaneous Pathology, 1996
- Dermatofibrosarcoma ProtuberansThe American Journal of Surgical Pathology, 1992
- Dermatofibrosarcoma protuberans with fibrosarcomatous areas. A clinicopathologic study of nine cases and a comparison with allied tumorsCancer, 1989
- Dermatofibrosarcoma protuberans: a clinicopathological and immunohistochemical study with a review of the literatureHistopathology, 1985
- Dermatofibrosarcoma protuberans: Study of fifty-six casesThe American Journal of Surgery, 1966