Universität zu Köln

Voth, H., Landsberg, J., Hinz, T., Wenzel, J., Bieber, T., Reinhard, G., Hoeller, T., Wendtner, C. -M. and Schmid-Wendtner, M. -H. (2011). Management of dermatofibrosarcoma protuberans with fibrosarcomatous transformation: an evidence-based review of the literature. J. Eur. Acad. Dermatol. Venereol., 25 (12). S. 1385 - 1392. HOBOKEN: WILEY. ISSN 1468-3083

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Abstract

Fibrosarcomatous transformation represents a rare event in dermatofibrosarcoma protuberans (DFSP) with unpredictable biological behaviour. No guidelines for the adequate treatment of patients with this rare neoplasm have been published. Herein, we present a comprehensive review of the literature comprising 157 patients with transformed DFSP focussing on surgical and adjuvant treatment modalities for this tumour. In the cohort examined, local recurrence occurred in 36% of cases and was significantly lower in patients treated by wide excision with margins =2 cm when compared with those treated with local excision without defined margins (P = 0.01). Consistently, negative margin status was associated with a lower recurrence rate when compared with positive or unknown margin status (P = 0.01). Distant metastases were detected in 13% of patients, which is significantly higher when compared with ordinary dermatofibrosarcoma protuberans. Systemic dissemination was preceded by local recurrence in 81% of cases, and is therefore strongly associated with tumour recurrence (P = 0.001). The present data confirm that wide excision with margins =2 cm represent the gold standard in the treatment of transformed dermatofibrosarcoma protuberans, and prevents recurrence as well as metastasis. When R0-resection is not feasible, adjuvant radiation should be considered for cases with incomplete resection or unknown surgical margins. Irresectable or metastatic transformed DFSP harbouring the COL1A1-PDGFB fusion gene should be treated with imatinib in the palliative setting or as an adjunctive treatment before surgery, although responses may be short-lasting.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Voth, H. UNSPECIFIED UNSPECIFIED UNSPECIFIED Landsberg, J. UNSPECIFIED UNSPECIFIED UNSPECIFIED Hinz, T. UNSPECIFIED UNSPECIFIED UNSPECIFIED Wenzel, J. UNSPECIFIED UNSPECIFIED UNSPECIFIED Bieber, T. UNSPECIFIED UNSPECIFIED UNSPECIFIED Reinhard, G. UNSPECIFIED UNSPECIFIED UNSPECIFIED Hoeller, T. UNSPECIFIED UNSPECIFIED UNSPECIFIED Wendtner, C. -M. UNSPECIFIED UNSPECIFIED UNSPECIFIED Schmid-Wendtner, M. -H. UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-484108
DOI:	10.1111/j.1468-3083.2011.04141.x
Journal or Publication Title:	J. Eur. Acad. Dermatol. Venereol.
Volume:	25
Number:	12
Page Range:	S. 1385 - 1392
Date:	2011
Publisher:	WILEY
Place of Publication:	HOBOKEN
ISSN:	1468-3083
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language MOHS MICROGRAPHIC SURGERY; WIDE LOCAL EXCISION; PROGNOSTIC-SIGNIFICANCE; SINGLE INSTITUTION; IMATINIB MESYLATE; AREAS; RECURRENCE; EMPHASIS; SARCOMAS; THERAPY Multiple languages Dermatology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/48410