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Table of Contents
Essential features | Etiology | Clinical features | Radiology description | Prognostic factors | Treatment | Gross description | Microscopic (histologic) description | Differential diagnosisCite this page: General. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/breastmalignantsarcomageneral.html. Accessed July 14th, 2017.
Essential features
- Rare, less than 1% of malignant breast tumors
- Represent < 5% of all sarcomas
- Treatment and behavior (metastases) are more like soft tissue sarcoma than like breast carcinoma
- May arise as primary tumors or secondary to radiation or chronic lymphedema
- Most common subtype is angiosarcoma
Etiology
- Primary breast sarcoma may be associated with genetic conditions such as Li-Fraumeni syndrome and neurofibromatosis type 1 (Surg Clin North Am 2008;88:451)
- Also secondary to radiation for breast carcinoma or other thoracic malignancy with an incidence of 0.03 - 0.2% and average latency period of 10 years (Cases J 2008;1:313)
Clinical features
- Most arise as a unilateral, rapidly enlarging palpable mass (Expert Rev Anticancer Ther 2014;14:705)
- Median age of diagnosis 49.5 years, although tendency to occur in younger age when primary sarcoma compared to sarcoma secondary to radiation (J Clin Pathol 2016;69:373)
- Lungs and liver are most common sites of metastases
Radiology description
- Unlike breast carcinoma, the most common finding is a large, oval, hypervascular mass with indistinct margins (AJR Am J Roentgenol 2012;198:W386)
Prognostic factors
- Favorable prognostic factors: non-menopausal status, no residual tumor following surgical resection (R0), non angiosarcoma subtype, grade 1 - 2 histology (Radiother Oncol 2007;85:355)
- Size < 5 cm has been shown to be the most favorable prognostic indicator of overall survival (Am J Surg 2008;196:559)
- Previous irradiation associated with poorer prognosis (Eur J Surg Oncol 2011;37:703)
- Angiosarcoma is the worst subtype
Treatment
- Complete surgical resection (R0)
- Axillary metastasis are rare and therefore axillary dissection is not indicated unless clinical evidence of nodal metastatses (Ann Oncol 2014;25:iii102)
- Adjuvant radiotherapy recommended in primary breast sarcoma > 5 cm, high grade and with positive resection margins (Expert Rev Anticancer Ther 2012;12:1045)
- Lack of evidence for using radiation in the neoadjuvant setting, and use is cautioned in radiation induced sarcomas
- Benefit of adjuvant chemotherapy is controversial (Surgery 1994;116:505, Clin Breast Cancer 2012;12:438, J Clin Oncol 2003;21:2583)
Gross description
- Generally large, median size of 5.25 cm
- Blue / purple skin discoloration may be seen in angiosarcoma
Microscopic (histologic) description
- Dependent on sarcoma subtype
Differential diagnosis
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