Breast malignant, males, children
Sarcoma
General

Author: Emily S. Reisenbichler, M.D. (see Authors page)

Revised: 14 June 2016, last major update June 2016

Copyright: (c) 2002-2016, PathologyOutlines.com, Inc.

PubMed Search: Breast sarcoma [title]

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
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
Gross Description
  • Generally large, median size of 5.25 cm
  • Blue / purple skin discoloration may be seen in angiosarcoma
Micro Description
  • Dependent on sarcoma subtype