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.