Breast Desmoid Tumor with Spectacular Evolution : A Case Report and Review of Current Treatment Options

Desmoid tumors or aggressive fibromatosis of the breast, are a rare entity, representing less than 0.2% of all primary breast tumors. The clinical presentation and evolution can mimic a malignant carcinoma, with the notable difference that a desmoid tumor cannot generate distant metastases. The aim of the treatment is to achieve local control of this tumor, which can be highly aggressive by deeply infiltrating surrounding structures, and frequently reoccurs after resection. Both the tumor and its treatment may cause significant morbidity, causing a real therapeutic challenge. We here report the case of a 63-year-old woman who underwent a tumorectomy for left breast cancer and developed six years later a large desmoid tumor in the same breast. First medically treated with selective estrogen receptor modulators and non-steroidal anti-inflammatory drugs, it progressed to an ulcerative exophytic and necrotic tumor requiring surgery. To our knowledge, this is the first description of such a spectacular evolution in the literature. After reporting on this uncommon evolution of a rare disease, we will provide a short review of current treatment options