Marcus J R, Few J W, Senger C, Reynolds M
Department of Pediatric Surgery, The Children's Memorial Hospital of Chicago, Northwestern University Medical School, IL 60611, USA.
J Pediatr Surg. 1998 Dec;33(12):1811-4. doi: 10.1016/s0022-3468(98)90292-5.
Dermatofibrosarcoma protuberans (DFSP) is a low-intermediate-grade cutaneous sarcoma that has a marked propensity for local recurrence after excision. The Bednar variant of this tumor is even less common and is distinguished histologically by the dispersal of melanin containing cells in an otherwise typical DFSP. Both are considered to be tumors of the third and forth decades of life, but both DFSP and the Bednar variant have been described in children. Until this report of a congenital Bednar tumor, only the DFSP has also been described in the neonate. The histopathology and surgical management of DFSP and Bednar tumors are outlined with emphasis on reported experience in the pediatric population. The surgical management of these lesions in children is based on numerous series in adults and the limited pediatric experience. The recommended treatment is wide excision with 3-cm margins of visibly uninvolved tissue and inclusion of superficial fascia.
隆突性皮肤纤维肉瘤（DFSP）是一种中低级别皮肤肉瘤，切除后极易局部复发。该肿瘤的贝德纳变异型更为罕见，组织学上的特征是在典型的DFSP中散在分布含黑色素细胞。二者均被认为是三四十岁人群的肿瘤，但DFSP和贝德纳变异型在儿童中也有报道。在这份先天性贝德纳肿瘤报告之前，仅新生儿DFSP有过描述。本文概述了DFSP和贝德纳肿瘤的组织病理学及手术治疗，重点介绍了儿科人群的报道经验。儿童这些病变的手术治疗基于成人的大量病例系列及有限的儿科经验。推荐的治疗方法是广泛切除，切缘距可见未受累组织3厘米，并包括浅筋膜。
