Abstract
Primary sarcoma of the breast is an extremely rare and heterogeneous disease. The rarity of this tumour limits most studies to small retrospective case reviews and case reports and has made clinicopathological study difficult. This article reviews the current literature on the diagnosis and management of breast sarcoma. The optimal treatment of breast sarcoma involves a multidisciplinary team prior to the initiation of treatment. Patients with tumours less than 5 cm that are easily resectable should undergo complete resection to the extent required to provide negative surgical margins. Negative surgical margins are more important for local recurrence and overall survival than the extent of surgical resection. Thus, neoadjuvant chemotherapy should be considered in order to shrink the tumour and help obtain negative surgical margins. Whether chemotherapy is indicated is primarily determined by tumour size. There is evidence that tumours larger than 5 cm are associated with an elevated risk of systemic failure and a poor prognosis. After surgical resection, patients with chemosensitive tumours should undergo additional adjuvant chemotherapy to treat micrometastatic disease. Radiation therapy should be used to improve local control in cases in which the tumour is larger than 5 cm and in cases with positive surgical margins. We propose to treat the patients according to the clinical practice guidelines in use for soft tissue sarcomas and address them to a reference centre for sarcoma. The appropriate treatment of breast sarcoma requires a multidisciplinary team approach necessitating experienced sarcoma surgeons, pathologists, radiotherapists and medical oncologists. Treating rare tumours in the same place should permit us to standardise pathological data and to include patients into multicentric radiotherapy or chemotherapy protocols to improve overall survival.
Similar content being viewed by others
References
Chelius M (1828) Teleangiektasie. Heidelberger Klin Ann 499:517
Müller J (1838) Über den feineren Bau und die Formen der krankhaften Geschwülste
Lee B, Pack G (1931) Giant intracanalicular myxoma: the so-called cystosarcoma phyllodes mammae of Johannes Muller. Am J Cancer 93:250–268
Schmidt GB (1887) Ueber das angiosarkom der mamma. Arch Klin Chir 36:421–427
Simon W (1912) Myeloische Chloro-Leukämie (Chlorom) unter dem Bilde eines malignen Mammatumors. Berl Klin Wochenschr 49:893–897
Pollard MSG et al (1990) Breast sarcoma. A clinicopathologic review of 25 cases. Cancer 66(5):941–944
Terrier PH et al (1989) Primary breast sarcoma: a review of 33 cases with immunohistochemistry and prognostic factors. Breast Cancer Res Treat 13(1):39–48
McGowan TS et al (2000) An analysis of 78 breast sarcoma patients without distant metastases at presentation. Int J Radiat Oncol Biol Phys 46(2):383–390
May DS, Stroup NE (1991) The incidence of sarcomas of the breast among women in the United States, 1973–1986. Plast Reconstr Surg 87(1):193–194
Fields RC et al (2008) Treatment and outcomes of patients with primary breast sarcoma. Am J Surg 196(4):559–561
Adem C et al (2004) Primary breast sarcoma: clinicopathologic series from the Mayo Clinic and review of the literature. Br J Cancer 91(2):237–241
Bousquet G et al (2007) Outcome and prognostic factors in breast sarcoma: a multicenter study from the rare cancer network. Radiother Oncol 85:355–361
Lattes R (1978) Sarcomas of the breast. Int J Radiat Oncol Biol Phys 4(7–8):705–708
McGregor GI, Knowling MA, Este FA (1994) Sarcoma and cystosarcoma phyllodes tumors of the breast: a retrospective review of 58 cases. Am J Surg 167(5):477–480
Confavreux C et al (2006) Sarcomas and malignant phyllodes tumours of the breast—a retrospective study. Eur J Cancer 42(16):2715–2721
Turner NC, Jones AL (2008) Management of breast cancer—Part I. BMJ 337:107–110
Lum YW, Jacobs L (2008) Primary breast sarcoma. Surg Clin N Am 88:559–570
Karlsson P et al (1998) Soft tissue sarcoma after treatment for breast cancer—a Swedish population-based study. Eur J Cancer 34(13):2068–2075
Huang J, Mackillop WJ (2001) Increased risk of soft tissue sarcoma after radiotherapy in women with breast carcinoma. Cancer 92(1):172–180
Taghian A et al (1991) Long-term risk of sarcoma following radiation treatment for breast cancer. Int J Radiat Oncol Biol Phys 21(2):361–367
Blanchard DK et al (2002) Radiation-induced breast sarcoma. Am J Surg 184(4):356–358
Blanchard DK et al (2003) Primary nonphylloides breast sarcomas. Am J Surg 186(4):359–361
Brady MS et al (1994) Post-treatment sarcoma in breast cancer patients. Ann Surg Oncol 1(1):66–72
Stewart FW, Treves N (1948) Lymphangiosarcoma in postmastectomy lymphedema: report of six cases in elephantiasis chirurgica. Cancer 1:64–81
McDivitt RW, Urban JA, Farrow JH (1967) Cystosarcoma phyllodes. Johns Hopkins Med J 120(1):33–45
Malkin D et al (1990) Germ line p53 mutations in a familial syndrome of breast cancer, sarcomas, and other neoplasms. Science 250(4985):1233–1238
Hollstein M et al (1994) p53 mutations at A:T base pairs in angiosarcomas of vinyl chloride-exposed factory workers. Carcinogenesis 15(1):1–3
Barrow BJ et al (1999) Role of radiotherapy in sarcoma of the breast—a retrospective review of the MD Anderson experience. Radiother Oncol 52(2):173–178
Berg JW (1961) Stromal sarcomas of the breast. A unified approach to connective tissue sarcomas other than cystosarcoma phyllodes. Cancer 15(2):418–424
Callery CD, Rosen PP, Kinne DW (1985) Sarcoma of the breast. A study of 32 patients with reappraisal of classification and therapy. Ann Surg 201(4):527–532
Christensen L et al (1988) Sarcomas of the breast: a clinico-pathological study of 67 patients with long term follow-up. Eur J Surg Oncol 14(3):241–247
Gutman H et al (1994) Sarcoma of the breast: implications for extent of therapy. The MD Anderson experience. Surgery 116(3):505–509
North JH et al (1998) Sarcoma of the breast: implications of the extent of local therapy. Am Surg 64(11):1059–1061
Pandey M et al (2004) Primary sarcoma of the breast. J Surg Oncol 87(3):121–125
Zelek L et al (2003) Prognostic factors in primary breast sarcomas: a series of patients with long-term follow-up. J Clin Oncol 21(13):2583–2588
McDivitt RW, Stewart FW, Berg JW (1968) Atlas of tumor pathology. Tumors of the breast. Armed Forces Institute of Pathology, Washington, DC
Shabahang M et al (2002) Surgical management of primary breast sarcoma. Am Surg 68(8):673–677
Ward RM, Evans HL (2006) Cystosarcoma phyllodes. A clinicopathologic study of 26 cases. Cancer 58(10):2282–2289
Pencavel TD, Hayes A (2009) Breast sarcoma—a review of diagnosis and management. Int J Surg 7(1):20–23
Foxcroft LM, Evans EB, Porter AJ (2007) Difficulties in the pre-operative diagnosis of phyllodes tumours of the breast: a study of 84 cases. Breast 16(1):27–37
Elson BC et al (1992) Fibrosarcoma of the breast: mammographic findings in five cases. Am J Roentgenol 158(5):993–995
Liberman L et al (1996) Benign and malignant phyllodes tumors: mammographic and sonographic findings. Radiology 198(1):121–124
Yang WT et al (2007) Mammary angiosarcomas: imaging findings in 24 patients. Radiology 242(3):725–734
Trent JC, Benjamin RS, Valero V (2001) Primary soft tissue sarcoma of the breast. Curr Treat Options Oncol 2(2):169–176
Tan PH (2005) Breast phyllodes tumours—morphology and beyond. Galloway Mem Lect 34(11):671–677
Esposito NN et al (2006) Phyllodes tumor: a clinicopathologic and immunohistochemical study of 30 cases. Arch Pathol Lab Med 130(10):1516–1520
Greene FL, Fritz AG, Balch CM (2002) AJCC cancer staging manual. Soft tissue sarcoma. Springer-Verlag, New York
Daigeler A et al (2009) Lymph node metastases in soft tissue sarcomas—a single center analysis of 1,597 patients. Langenbecks Arch Surg 394(2):321–329
Bakheet SM et al (1998) F-18 FDG whole-body positron emission tomography scan in primary breast sarcoma. Clin Nucl Med 23(9):604–608
Smola MG et al (1993) The impact of resection margins in the treatment of primary sarcomas of the breast. A clinicopathological study of 8 cases with review of literature. Eur J Surg Oncol 19(1):61–69
Johnstone PAS et al (1993) Primary soft tissue sarcomas of the breast: local-regional control with post-operative radiotherapy. Int J Radiat Oncol Biol Phys 27(3):671–675
Moore MP, Kinne DW (1996) Breast sarcoma. Surg Clin N Am 76(2):383–392
Monroe AT, Feigenberg SJ, Mendenhall NP (2003) Angiosarcoma after breast-conserving therapy. Cancer 97(8):1832–1840
Patel SR et al (1998) Results of two consecutive trials of dose-intensive chemotherapy with doxorubicin and ifosfamide in patients with sarcomas. Am J Clin Oncol 21(3):317–321
Patel SR et al (1997) High-dose ifosfamide in bone and soft tissue sarcomas: results of phase II and pilot studies—dose-response and schedule dependence. J Clin Oncol 15(6):2378–2384
Brennan M, Casper E, Harrison L (2008) Soft tissue sarcoma. In: DeVita JV, Hellman S, Rosenberg S (eds) Cancer: principles and practice of oncology. Lippincott Williams and Wilkins, Philadelphia, pp 1731–1852
Thalhammer M et al (2006) Hyperthermia as a therapeutic option in recurrent breast sarcoma. Eur J Surg Oncol 32(S1):64
Issels RD et al (1990) Ifosfamide plus etoposide combined with regional hyperthermia in patients with locally advanced sarcomas: a phase II study. J Clin Oncol 8(11):1818–1829
Issels RD (2008) Regional hyperthermia in high-risk soft tissue sarcomas. Curr Opin Oncol 20(4):438–443
Issels RD et al (2010) Neo-adjuvant chemotherapy alone or with regional hyperthermia for localised high-risk soft-tissue sarcoma: a randomised phase 3 multicentre study. Lancet Oncol. doi:10.1016/S1470-2045(10)70071-1
Ciatto S et al (1992) Sarcomas of the breast: a multicenter series of 70 cases. Neoplasma 39(6):375–379
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Al-Benna, S., Poggemann, K., Steinau, HU. et al. Diagnosis and management of primary breast sarcoma. Breast Cancer Res Treat 122, 619–626 (2010). https://doi.org/10.1007/s10549-010-0915-y
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10549-010-0915-y