Darrier和Ferrand皮纤维肉瘤:复发特征:病例报告(丹吉尔-泰图安-胡塞马大学医院的经验)

Dehhaze Adil, Mai Aicha, Barij Hamza, Mahioui Mimoun, Bouazza Omar, Taybi Otmane, Daghouri Nada-Imane, Labbaci Rim, Diher Issam, Echmili Mouad, Tazi Hanae
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引用次数: 0

摘要

导言:达里尔和费朗皮肤纤维肉瘤(DFSP)是一种罕见的皮肤肿瘤,具有局部侵袭性和高潜伏性的特点。其预后主要取决于治疗的质量。观察和结果:本文报告了丹吉尔整形、修复和美容外科治疗的一例年轻患者的病例,该患者患有 Darier 和 Ferrand 皮纤维肉瘤,手术切除后复发 5 次,表面边缘 5 厘米,深部有健康的解剖屏障。在解剖病理证实切除术具有癌变性质后,对实质缺失(LOS)进行了覆盖,主要包括植皮。尽管在第 4 次复发时进行了放疗,但第 5 次又迅速复发。讨论:这种类型的肉瘤在临床、组织学和进化上存在很大的多态性,导致发病率被低估,这也是诊断延误的原因。复发很频繁,通常是局部复发。最初的广泛手术切除是参考治疗方法,直接影响 DFSP 的预后。根据多项研究,复发可能取决于手术切除的质量,但也可能归因于遗传或环境因素,或肉瘤组织学亚型的变化。结论Darier Ferrand 是一种不常见的局部恶性肿瘤,其演变风险主要取决于初次切除的质量。初诊手术做得好的患者治愈的几率明显高于抢救性手术。因此,肿瘤切除必须广泛而深入,并牺牲深部的健康屏障。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Dermatofibrosarcoma of Darrier and Ferrand: Recurrency Character: A Case-Report (Experience of the University Hospital Tangier-Tetouan-Al Hoceima)
Introduction: Dermatofibrosarcoma of Darier and Ferrand (DFSP) is a rare skin tumor characterized by its local aggressiveness and high potential. Its prognosis depends essentially on the quality of its management. Observation and Results: In this article, we report the case of a young patient treated at the Department of Plastic, Reconstructive and Aesthetic Surgery, Tangier, for a Darier and Ferrand dermatofibrosarcoma that recurred five times after surgical excision, with a 5-cm margin on the surface and a healthy anatomical barrier at depth. Coverage of the loss of substance (LOS) was performed after anatomopathological confirmation of the carcinological nature of the excision, and essentially involved skin grafting. Despite a course of radiotherapy prescribed at the 4th recurrence, a 5th rapid recurrence was noted. Discussion: The great clinical, histological and evolutionary polymorphism of this type of sarcoma results in an underestimated incidence and explains the delay in diagnosis. Recurrences are frequent, and usually local. Initial wide surgical excision is the reference treatment, and directly conditions the prognosis of DFSP. According to several studies, recurrence may depend on the quality of surgical excision, but could also be attributed to genetic or environmental factors, or to variations in the histological subtype of the sarcoma. Conclusion: Darier Ferrand is an uncommon tumor with local malignancy, whose evolutionary risk depends mainly on the quality of the initial excision. The chances of cure in the case of well performed primary surgery are significantly higher than in the case of salvage surgery. Tumor excision must the refore be wide and deep, with sacrifice of a healthy barrier at depth.
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