Juvenile fibromatosis: hormonal receptors

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.