Medical treatment of mammary desmoid-type fibromatosis: which benefit?

Abstract

BackgroundBreast fibromatosis is a rare disease characterized by monoclonal fibroblast proliferation. It has no ability to metastasize but has a high local recurrence rate and often infiltrates surrounding tissues. Surgical treatment is the reference, but recently, new targeted therapies have emerged. We report an original case of a patient with breast fibromatosis who received exclusive medical treatment. Our aim was to analyze these treatments based on the clinical and radiological outcome, iatrogenic effects, and pharmacological action.Case presentationWe report the case of a 19-year-old woman who developed a desmoid-type fibromatosis of the lower inner quadrant of the right breast, measuring 50 × 25 mm (i.e., a volume of 27.4 cm3). Initial surgery was not possible because of potential esthetic and functional prejudice. Thus, she had an exclusive medical treatment including several lines: NSAIDs with tamoxifen and triptorelin, followed by sorafenib, then interferon α2b, and finally sunitinib. With tyrosine-kinase inhibitors (TKIs) (sunitinib), a significant partial response was observed (57% reduction of the maximal tumoral volume). For each treatment, we provided the clinical and radiological outcome in association with known pharmacological action.ConclusionsTKI had been an interesting alternative option to initial surgery, providing at least a partial response and potentially allowing less mutilating surgery. However, no pharmacological mechanism can unequivocally explain TKI efficacy. In general, breast fibromatosis should be treated along with oncologist and interventional radiologists in a trans-disciplinary modality, thus offering an adapted treatment for this particular desmoid-type fibromatosis localization.

Breast fibromatosis: Making the case for primary vs secondary subtypes

“…As supported by authors Povoski et al in 2006, a patient with recurrent fibromatosis of the breast required a radical excision including chest wall and rib resection. This approach is acceptable to obtain loco‐regional control, and it also provided the patient symptomatic relief due to chest wall pain . Furthermore, our patient presented previously also required resection of the chest wall and ribs, which resulted in negative margins and without significant morbidity.…”

“…Mammographic findings mimic breast carcinoma and are often indistinguishable; however, calcifications are an unusual clinical finding with this entity. Additionally, ultrasound findings may show a well‐circumscribed mass that resembles a fibroadenoma, which can result in inadequate resections, which is an important pitfall to avoid . Pain is often a finding in advanced disease when the chest wall is involved.…”

“…As far as medical treatment of fibromatosis, this should be reserved for those with unresectable disease or the presence of persistently positive margins. Hormonal therapy with tamoxifen has been described with some success in several series (up to 20% of tumors responding) . There has also been some success with tyrosine kinase inhibitors such as imatinib .…”

“…Hormonal therapy with tamoxifen has been described with some success in several series (up to 20% of tumors responding) . There has also been some success with tyrosine kinase inhibitors such as imatinib . However, these treatments have limited efficacy and take several months for the tumor to have a response .…”

“…There has also been some success with tyrosine kinase inhibitors such as imatinib . However, these treatments have limited efficacy and take several months for the tumor to have a response . Radiation treatment can also be considered in advanced cases, positive margins, or for tumor which abuts vital structures .…”