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Titre du document / Document title

Dermatofibrosarcoma protuberans : A clinicopathologic analysis of patients treated and followed at a single institution

Auteur(s) / Author(s)

BOWNE Wilbur B. (1) ; ANTONESCU Cristina R. (2) ; LEUNG Denis H. Y. (3) ; KATZ Steven C. (1) ; HAWKINS William G. (1) ; WOODRUFF James M. (2) ; BRENNAN Murray F. (1) ; LEWIS Jonathan J. (1) ;

Affiliation(s) du ou des auteurs / Author(s) Affiliation(s)

(1) Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, New York 10021, ETATS-UNIS
(2) Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, New York 10021, ETATS-UNIS
(3) Department of Biostatistics, Memorial Sloan-Kettering Cancer Center, New York, New York 10021, ETATS-UNIS

Résumé / Abstract

BACKGROUND. Despite optimal surgical therapy for patients with dermatofibrosarcoma protuberans (DFSP), some patients still continue to develop local recurrence. The authors' objective was to identify and analyze clinicopathologic factors for disease free survival in a large group of patients who were followed prospectively at a single institution. METHODS. Prospectively collected data and pathology slides were available for review from 159 patients with primary or recurrent DFSP who underwent treatment between July 1950 and July 1998. The study group was comprised of patients with either the classic form of DFSP or the fibrosarcomatous high grade variant of DFSP (FS-DFSP). Patient, tumor, pathologic, and treatment factors were analyzed using the log rank test for univariate influence and Cox regression analysis for multivariate influence. Local recurrence free survival was determined by the Kaplan-Meier actuarial method. RESULTS. Of the 159 patients who comprised the current study group, 134 (84%) had the classic form of DFSP. The FS-DFSP variant was found in the remaining 25 patients (16%). The overall 5-year local recurrence free survival rate was 75%, with a median follow-up of 4.75 years. The 5-year recurrence free survival rate for each group was 81% and 28%, respectively. On univariate analysis, age > 50 years, very close (< 1 mm) to positive microscopic margins, FS-DFSP variant, high mitotic rate, and increased cellularity were unfavorable prognostic factors. Multivariate analysis determined very close (< 1 mm) to positive microscopic margins and FS-DFSP variant to be independent adverse prognostic factors. For the 34 patients who developed a recurrence after surgical resection (21%), the median time to local recurrence was 32 months. Of the patients in this group, two died from metastatic disease. CONCLUSIONS. The prognosis after surgical resection with negative and sometimes positive microscopic margins for patients with DFSP is very good. However, increased age, high mitotic index, and increased cellularity are predictors of poor clinical outcome. The FS-DFSP variant represents a much more aggressive tumor with metastatic potential. Patients who are treated with curative intent for FS-DFSP should undergo aggressive attempts at complete surgical resection. Patients with recurrent classic DFSP without evidence of adverse prognostic features may benefit from conservative management, especially in the setting of potentially unresectable disease.

Revue / Journal Title

Cancer    ISSN  0008-543X   CODEN CANCAR 

Source / Source

2000, vol. 88, no12, pp. 2711-2720 (26 ref.)

Langue / Language

Anglais

Editeur / Publisher

Wiley-Blackwell, Hoboken, NJ, ETATS-UNIS  (1948) (Revue)

Mots-clés anglais / English Keywords

Dermatofibrosarcoma protuberans

;

Surgical resection

;

Treatment

;

Prognosis

;

Human

;

Malignant tumor

;

Skin disease

;

Sarcoma

;

Surgery

;

Mots-clés français / French Keywords

Dermatofibrosarcome Darier Ferrand

;

Résection chirurgicale

;

Traitement

;

Pronostic

;

Homme

;

Tumeur maligne

;

Peau pathologie

;

Sarcome

;

Chirurgie

;

Mots-clés espagnols / Spanish Keywords

Dermatofibrosarcoma Darier Ferrand

;

Resección quirúrgica

;

Tratamiento

;

Pronóstico

;

Hombre

;

Tumor maligno

;

Piel patología

;

Sarcoma

;

Cirugía

;

Localisation / Location

INIST-CNRS, Cote INIST : 2701, 35400009134926.0080

Nº notice refdoc (ud4) : 1476351



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