Rare Disease: Breast Desmoid-Type Fibromatosis.

Abstract

The article presents a rare form of pathological neoplasms — breast desmoid type fibromatosis. There are etiology and pathogenesis of the disease, clinical and morphological features, diagnostics and tactics of treatment of the disease. Widely covered clinical examples from the practice of a surgeon-oncologist. Desmoid fibromatosis (DF) is an aggressive non-metastatic mesenchymal formation that arises from musculoponeurotic structures. DF accounts for 0.03 % to 0.1 % of solid tumors and 3 % of mesenchymal tumors. Among breast pathology, this neoplasm is even rarer (0.2 %). Due to the rarity of such pathology, clinicians are not always able to correctly interpret the diagnostic picture and recognize the disease. The etiology and pathogenesis of the disease are also not fully understood, and therefore there are no standards for the treatment of desmoid fibromatosis. Surgical treatment remains the main one. To reduce the frequency of recurrence of the disease, the surgeon needs to achieve clean edges of the resection. Radiation therapy, hormone therapy, targeted therapy, and chemotherapy may additionally be considered as an alternative type of treatment. When the clean edges of the resection are reached, strict dynamic monitoring of pa is considered as further treatment.