Optimal management of sarcomas of the breast: an update

Abstract

Breast sarcomas are rare mesenchymal-derived breast tumors. The small number of patients, the different histological subtypes, and the variation in clinical practice impairs the ability to draw firm practice recommendations. Patient management is often extrapolated from other soft tissue sarcomas, mostly of the extremities in which more clinical data is available. Surgical resection with negative margins is the goal of treatment, irrespective of the surgical procedure; the implication of radiation and chemotherapy is variable. Further advances in treatment should follow the assembly of breast sarcoma patients in specific cancer networks in specialized sarcoma referral centers. The characterization of molecular pathways active in tumorogenesis of these tumors may pave the way for the application of novel therapeutic agents.

Keywords::

• breast sarcoma
• chemotherapy
• radiation therapy
• soft tissue sarcoma
• surgery

Financial & competing interests disclosure

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

No writing assistance was utilized in the production of this manuscript.

Key issues

• Tumor breast sarcomas are rare mesenchymal-derived tumors with various histological subtypes.

• Most of the data concerning the presentation, diagnosis and management of these tumors stem from relatively small series; hence, extrapolation from other soft tissue sarcoma is used to bridge the existent knowledge gaps.

• The mainstay of treatment is surgical resection with negative margins, with no predilection for mastectomy over lumpectomy (with the exception of large tumor: breast ratio and ill-defined angiosarcomas). When possible, reoperation is advised for R1/R2 resection in order to achieve negative microscopic margins.

• Adjuvant radiation therapy may be attempted in high-risk cases (positive margins, large and high-grade tumors). The use of adjuvant chemotherapy is debatable and should be considered individually by a specialized team.

• Centralization of patients in referral centers and formation of worldwide cancer networks should be implied to advance both research and care of breast sarcomas.

Notes