Maddalozzo J, Tenta L T, Hutchinson L R, Crawford S E, Morse D S
Dept. of Otolaryngology (Head and Neck Surgery), Rush-Presbyterian-St. Luke's Medical Center, Chicago, IL.
Int J Pediatr Otorhinolaryngol. 1993 Jan;25(1-3):191-9. doi: 10.1016/0165-5876(93)90053-6.
Aggressive juvenile fibromatosis, though allegedly a benign process, is as frustrating to manage as it is perplexing to comprehend. The treatment is primarily surgical, with chemotherapy and radiation therapy recently finding support as adjuncts in selected circumstances. Though there is no agreement regarding the etiology of fibromatosis, many have suspected hormonal or traumatic influences. There has been historical, clinical and experimental data demonstrating that fibromatosis seems to be under the influence of estrogen. There have also been anecdotal reports that this tumor has regressed with the use of tamoxifen. To our knowledge, no one has tested these tissues for the presence of estrogen/progesterone receptors. Recently, over a short period of time, we at Children's Memorial Hospital, Chicago, have treated four youngsters with this disorder. All operative specimens were submitted for estrogen and progesterone assays. Although these cases were indistinguishable on histologic examination, two of the four exhibited the presence of estrogen/progesterone receptors. We propose, that from these observations, there potentially may be derived a histochemical classification based upon the presence or absence of estrogen/progesterone receptors. This would serve as an added reference in the definition and treatment of this disease. Should hormonal receptors be present, agents such as tamoxifen conceivably could be employed as part of a post-operative maintenance regimen similar to those protocols applied in the management of hormonally responsive breast cancer.
侵袭性青少年纤维瘤病尽管据称是一种良性病变，但其治疗管理令人沮丧，理解起来也令人困惑。治疗主要是手术治疗，化疗和放射治疗最近在某些特定情况下作为辅助治疗得到了支持。尽管关于纤维瘤病的病因尚无定论，但许多人怀疑其与激素或创伤因素有关。有历史、临床和实验数据表明，纤维瘤病似乎受雌激素影响。也有一些轶事报道称，使用他莫昔芬后这种肿瘤会消退。据我们所知，没有人对这些组织进行过雌激素/孕激素受体检测。最近，在短时间内，我们芝加哥儿童纪念医院治疗了4名患有这种疾病的青少年。所有手术标本都送去进行雌激素和孕激素检测。尽管这些病例在组织学检查上难以区分，但4例中有2例显示存在雌激素/孕激素受体。我们认为，基于这些观察结果，有可能得出一种基于雌激素/孕激素受体存在与否的组织化学分类方法。这将为这种疾病的定义和治疗提供额外的参考。如果存在激素受体，那么诸如他莫昔芬之类的药物可以设想作为术后维持治疗方案的一部分来使用，类似于应用于激素反应性乳腺癌管理的那些方案。
