Dermatofibrosarcoma protuberans: Review of 20-years experience

被引:22
作者
Ruiz-Tovar J. [1 ,4 ]
Guarino M.F. [2 ]
Callejas M.E.R. [3 ]
Velardo A.A. [1 ]
Bermejo J.A. [1 ]
Navarro L.C. [1 ]
机构
[1] Department of Surgery, University Hospital Ramón y Cajal, Madrid
[2] Department of Dermatology, University Hospital Ramón y Cajal, Madrid
[3] Department of Pathology, University Hospital Ramón y Cajal, Madrid
[4] 28002 Madrid, C/Corazón de María, 64
关键词
Dermatofibrosarcoma protuberans; Locally aggressive tumour; Softtissue sarcoma; storiform pattern;
D O I
10.1007/s12094-006-0067-z
中图分类号
学科分类号
摘要
Introduction: Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue neoplasm with low-intermediate grade of malignancy. It is a locally aggressive tumour with a high recurrence rate. Surgical excision with adequate margins is the main treatment. Materials and methods: We describe the clinicopathological features of 21 cases of DFSP. The mean size of the lesions was 5.6 cm, mostly located in trunk. 61.9% of the cases underwent sugical excision without previous biopsy. 52.4% of the patients presented positive margins, that required surgical extension. Results: The recurrence rate was 28.6% (6 cases), five of them local recurrences treated with new surgical excision with wide margins. Median period free of illness was 52.5 months. Medium follow-up period was 55.25 months. Conclusions: DFSP has a locally high recurrence rate, that has been associated to inadequate surgical margins. The histological knowledge preoperatively would permit surgical excision with adequate margins, and probably this could reduce the recurrence rate. Radiotherapy could avoid the surgical extension of margins in these cases with positive ones. Chemotherapy could be indicated in metastasic cases. Most recurrences appear in the first 3 years, but it is important a long-term follow-up of these patients. © FESEO 2006.
引用
收藏
页码:606 / 610
页数:4
相关论文
共 11 条
[1]  
Chang C.K., Jacobs I.A., Salti G.I., Outcomes of surgery for dermatofibrosarcoma protuberans, J Clin Oncol, 14, pp. 2565-2569, (1996)
[2]  
Gloster Jr. H.M., Dermatofibrosarcoma protuberans, J Am Acad Dermatol, 55, pp. 375-376, (1996)
[3]  
Lindner N.J., Scarborough M.T., Powell G.J., Spanier S., Enneking W.F., Revision surgery in dermatofibroma protuberans of the trunk and extremities, Eur J Surg Oncol, 25, pp. 392-397, (1999)
[4]  
Abenoza P., Lillemoe T., CD34 and factor XIIIa in the differential diagnosis of dermatofibroma and dermatofibrosarcoma protuberans, Am J Dermatopathol, 15, 5, pp. 429-434, (1993)
[5]  
Bowne W.B., Antonescu C.R., Leung D.H., Et al., Dermatofibrosarcoma protuberans. A clinicopa thologic analysis of patients treated and followed at a single institution, Cancer, 88, pp. 2711-2720, (2000)
[6]  
Rutgers E.J., Kroon B.B., Albus-Lutter C.E., Gortzak E., Dermatofibrosarcoma protuberans: Treatment and prognosis, Eur J Surg Oncol, 18, pp. 241-248, (1992)
[7]  
Enzinger F.M., Weiss S.W., Fibrohistiocytic tumors of intermediate malignancy, En: Stamathis G. Soft Tissue Tumors, pp. 252-268, (1988)
[8]  
Mendenhall W.M., Zlotecki R.A., Scarborough M.T., Dermatofibrosarcoma protuberans, Cancer, 101, 11, pp. 2503-2508, (2004)
[9]  
Ballo M.T., Zagars G.K., Pisters P., Pollack A., The role of radiation therapy in the management of dermatofibrosarcoma protuberans, Int J Radiat Oncol Biol Phys, 40, pp. 823-827, (1998)
[10]  
Rubin B.P., Schuetze S.M., Eary J.F., Et al., Molecular targeting of Platelet-Derived Growth Factor B by Imatinib Mesylate in a patient with dermatofibrosarcoma protuberans, J Clin Oncol, 20, 17, pp. 3586-3591, (2002)