Grange F, Avril M F, Margulis A, Duvillard P
Service de Dermatologie, Institut Gustave-Roussy, Villejuif.
Ann Dermatol Venereol. 1993;120(10):679-83.
We report the case of a 19 year-old female patient who consulted for a recurrent desmoid tumor of the back which had initially developed during childhood. Its clinical aspect was reminiscent of a localized subcutaneous tumor, but surgical exploration revealed multiple ramified extensions spreading over a wide area deep into the underlying muscular tissue. In spite of two attempts at wide surgery, complete resection was not achieved. EAD tumors are a distinct category between fibromatosis and fibrosarcoma. There is a direct contrast between the benign histological structure and the lack of a metastatic potential, and the fact that they are highly aggressive locally with a great proportion of relapses occurring after resection. Prognosis, which is good in terms of survival, is determinant in the choice of treatment which must remain conservative in the majority of cases. Wide, non mutilating surgery offers the best chances of cure without sequelae. When surgery may be mutilating, 50-60 Gy of radiotherapy (RT) is a good alternative and affords a high rate of complete regression over a long period of time. The value of adjuvant RT after surgical resection has yet to be determined. Hormonotherapy and other drugs are currently being evaluated.
我们报告了一例19岁女性患者，她因背部复发性硬纤维瘤前来就诊，该肿瘤最初在儿童期就已出现。其临床表现类似于局限性皮下肿瘤，但手术探查发现多个分支状延伸，广泛深入到下方的肌肉组织。尽管进行了两次广泛手术尝试，但仍未实现完全切除。硬纤维瘤样肿瘤是纤维瘤病和纤维肉瘤之间的一个独特类别。其良性组织学结构与无转移潜能之间存在直接对比，而且它们在局部具有高度侵袭性，大部分复发发生在切除术后。就生存而言预后良好，这在治疗选择中起决定性作用，在大多数情况下治疗必须保持保守。广泛的、不致残的手术提供了最佳的治愈机会且无后遗症。当手术可能致残时，则50 - 60 Gy的放疗（RT）是一个不错的选择，并且能在很长一段时间内实现高比例的完全消退。手术切除后辅助放疗的价值尚未确定。目前正在评估激素疗法和其他药物。
