Breast sarcoma: Treatment
- Rashmi Chugh, MD
Rashmi Chugh, MD
- Associate Professor of Medical Oncology
- University of Michigan Medical School
- Michael S Sabel, MD
Michael S Sabel, MD
- Associate Professor of Surgery
- University of Michigan Medical School
- Mary Feng, MD
Mary Feng, MD
- Associate Professor
- University of California at San Francisco
- Section Editors
- Robert Maki, MD, PhD
Robert Maki, MD, PhD
- Section Editor — Bone and Soft Tissue Tumors
- Professor of Medicine
- Monter Cancer Center
- Hofstra-Northwell School of Medicine
- Daniel F Hayes, MD
Daniel F Hayes, MD
- Section Editor — Breast Cancer
- Professor of Medicine
- University of Michigan School of Medicine
- Anees B Chagpar, MD, MSc, MA, MPH, MBA, FACS, FRCS(C)
Anees B Chagpar, MD, MSc, MA, MPH, MBA, FACS, FRCS(C)
- Section Editor — Breast Surgery
- Associate Professor, Department of Surgery
- Yale University School of Medicine
- Thomas F DeLaney, MD
Thomas F DeLaney, MD
- Section Editor — Bone and Soft Tissue Tumors
- Professor of Radiation Oncology
- Harvard Medical School
Breast sarcomas are rare, histologically heterogeneous nonepithelial malignancies that arise from the connective tissue within the breast. They can develop de novo (primary), after radiation therapy (RT), or in the setting of chronic lymphedema of the arm or breast (therapy related, secondary). Although the clinical features of breast sarcoma mimic those of breast carcinoma in some ways, therapy and prognosis differ dramatically.
Given the rarity of breast sarcomas, there are no prospective randomized trials to guide therapy. Treatment principles have been derived from small retrospective case reviews of breast sarcomas and extrapolated from studies of non-breast soft tissue sarcomas of the extremity and chest wall since clinical behavior, histology, and prognosis are similar.
As with soft tissue sarcomas arising in other areas of the body, a multidisciplinary approach at an experienced center involving surgical, radiation, and medical oncologists is preferred . In general, the choice of treatment is influenced by stage, histologic grade, and tumor size.
Surgery represents the only potentially curative modality for breast sarcomas. The type and extent of the operation is based upon both the size of the tumor and the size of the breast, as well as histology:
●An adequate resection margin is the single most important determinant of long-term survival with breast sarcomas [2-4]. For larger tumors (ie, those >5 cm), the overall cosmetic result is often better with a mastectomy and reconstruction than with lumpectomy. Deep seated tumors, which are close to or involve the chest wall, may require en bloc resection of the chest wall [3,5,6]. With the exception of angiosarcomas, the majority of primary breast sarcomas are not multicentric , and negative surgical margins are more important than the extent of surgical resection [2,7-9]. Breast angiosarcomas (primary or therapy-related) often affect a much larger field of the breast or chest wall than anticipated, and mastectomy is the standard treatment [10,11].
- Wiklund T, Huuhtanen R, Blomqvist C, et al. The importance of a multidisciplinary group in the treatment of soft tissue sarcomas. Eur J Cancer 1996; 32A:269.
- Zelek L, Llombart-Cussac A, Terrier P, et al. Prognostic factors in primary breast sarcomas: a series of patients with long-term follow-up. J Clin Oncol 2003; 21:2583.
- Pandey M, Mathew A, Abraham EK, Rajan B. Primary sarcoma of the breast. J Surg Oncol 2004; 87:121.
- Shabahang M, Franceschi D, Sundaram M, et al. Surgical management of primary breast sarcoma. Am Surg 2002; 68:673.
- North JH Jr, McPhee M, Arredondo M, Edge SB. Sarcoma of the breast: implications of the extent of local therapy. Am Surg 1998; 64:1059.
- Lum YW, Jacobs L. Primary breast sarcoma. Surg Clin North Am 2008; 88:559.
- McGowan TS, Cummings BJ, O'Sullivan B, et al. An analysis of 78 breast sarcoma patients without distant metastases at presentation. Int J Radiat Oncol Biol Phys 2000; 46:383.
- Moore MP, Kinne DW. Breast sarcoma. Surg Clin North Am 1996; 76:383.
- Trent II JC 2nd, Benjamin RS, Valero V. Primary soft tissue sarcoma of the breast. Curr Treat Options Oncol 2001; 2:169.
- Chapelier AR, Bacha EA, de Montpreville VT, et al. Radical resection of radiation-induced sarcoma of the chest wall: report of 15 cases. Ann Thorac Surg 1997; 63:214.
- Scow JS, Reynolds CA, Degnim AC, et al. Primary and secondary angiosarcoma of the breast: the Mayo Clinic experience. J Surg Oncol 2010; 101:401.
- Monroe AT, Feigenberg SJ, Mendenhall NP. Angiosarcoma after breast-conserving therapy. Cancer 2003; 97:1832.
- Al-Benna S, Poggemann K, Steinau HU, Steinstraesser L. Diagnosis and management of primary breast sarcoma. Breast Cancer Res Treat 2010; 122:619.
- Rosen, PP. Sarcoma. In: Rosen's Breast Pathology, Rosen, PP (Eds), Lippincott Williams and Wilkins, Philadelphia 2001. p.813.
- Sher T, Hennessy BT, Valero V, et al. Primary angiosarcomas of the breast. Cancer 2007; 110:173.
- Barrow BJ, Janjan NA, Gutman H, et al. Role of radiotherapy in sarcoma of the breast--a retrospective review of the M.D. Anderson experience. Radiother Oncol 1999; 52:173.
- Gullett NP, Delman K, Folpe AL, Johnstone PA. National surgical patterns of care: regional lymphadenectomy of breast sarcomas. Am J Clin Oncol 2007; 30:461.
- Gutman H, Pollock RE, Ross MI, et al. Sarcoma of the breast: implications for extent of therapy. The M. D. Anderson experience. Surgery 1994; 116:505.
- Fong Y, Coit DG, Woodruff JM, Brennan MF. Lymph node metastasis from soft tissue sarcoma in adults. Analysis of data from a prospective database of 1772 sarcoma patients. Ann Surg 1993; 217:72.
- Terrier P, Terrier-Lacombe MJ, Mouriesse H, et al. Primary breast sarcoma: a review of 33 cases with immunohistochemistry and prognostic factors. Breast Cancer Res Treat 1989; 13:39.
- Bousquet G, Confavreux C, Magné N, et al. Outcome and prognostic factors in breast sarcoma: a multicenter study from the rare cancer network. Radiother Oncol 2007; 85:355.
- McGregor GI, Knowling MA, Este FA. Sarcoma and Cystosarcoma phyllodes tumors of the breast--a retrospective review of 58 cases. Am J Surg 1994; 167:477.
- Kaklamanos IG, Birbas K, Syrigos KN, et al. Breast angiosarcoma that is not related to radiation exposure: a comprehensive review of the literature. Surg Today 2011; 41:163.
- Fields RC, Aft RL, Gillanders WE, et al. Treatment and outcomes of patients with primary breast sarcoma. Am J Surg 2008; 196:559.
- Nascimento AF, Raut CP, Fletcher CD. Primary angiosarcoma of the breast: clinicopathologic analysis of 49 cases, suggesting that grade is not prognostic. Am J Surg Pathol 2008; 32:1896.
- Blanchard DK, Reynolds CA, Grant CS, Donohue JH. Primary nonphylloides breast sarcomas. Am J Surg 2003; 186:359.
- Johnstone PA, Pierce LJ, Merino MJ, et al. Primary soft tissue sarcomas of the breast: local-regional control with post-operative radiotherapy. Int J Radiat Oncol Biol Phys 1993; 27:671.
- Fury MG, Antonescu CR, Van Zee KJ, et al. A 14-year retrospective review of angiosarcoma: clinical characteristics, prognostic factors, and treatment outcomes with surgery and chemotherapy. Cancer J 2005; 11:241.
- Pollard SG, Marks PV, Temple LN, Thompson HH. Breast sarcoma. A clinicopathologic review of 25 cases. Cancer 1990; 66:941.
- Rosen PP, Kimmel M, Ernsberger D. Mammary angiosarcoma. The prognostic significance of tumor differentiation. Cancer 1988; 62:2145.
- Depla AL, Scharloo-Karels CH, de Jong MA, et al. Treatment and prognostic factors of radiation-associated angiosarcoma (RAAS) after primary breast cancer: a systematic review. Eur J Cancer 2014; 50:1779.
- Sheth GR, Cranmer LD, Smith BD, et al. Radiation-induced sarcoma of the breast: a systematic review. Oncologist 2012; 17:405.
- Erel E, Vlachou E, Athanasiadou M, et al. Management of radiation-induced sarcomas in a tertiary referral centre: a review of 25 cases. Breast 2010; 19:424.
- Feigenberg SJ, Mendenhall NP, Reith JD, et al. Angiosarcoma after breast-conserving therapy: experience with hyperfractionated radiotherapy. Int J Radiat Oncol Biol Phys 2002; 52:620.
- Palta M, Morris CG, Grobmyer SR, et al. Angiosarcoma after breast-conserving therapy: long-term outcomes with hyperfractionated radiotherapy. Cancer 2010; 116:1872.
- Pervaiz N, Colterjohn N, Farrokhyar F, et al. A systematic meta-analysis of randomized controlled trials of adjuvant chemotherapy for localized resectable soft-tissue sarcoma. Cancer 2008; 113:573.
- Torres KE, Ravi V, Kin K, et al. Long-term outcomes in patients with radiation-associated angiosarcomas of the breast following surgery and radiotherapy for breast cancer. Ann Surg Oncol 2013; 20:1267.
- Lagrange JL, Ramaioli A, Chateau MC, et al. Sarcoma after radiation therapy: retrospective multiinstitutional study of 80 histologically confirmed cases. Radiation Therapist and Pathologist Groups of the Fédération Nationale des Centres de Lutte Contre le Cancer. Radiology 2000; 216:197.
- Confavreux C, Lurkin A, Mitton N, et al. Sarcomas and malignant phyllodes tumours of the breast--a retrospective study. Eur J Cancer 2006; 42:2715.
- Casali PG, Blay JY, ESMO/CONTICANET/EUROBONET Consensus Panel of experts. Soft tissue sarcomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol 2010; 21 Suppl 5:v198.
- Thalhammer, M, et al. Hyperthermia as a therapeutic option in recurrent breast sarcoma.Eur J Surg Oncol 2006; 32(S1):S64.Data presented at the 13th Congress of the European Society of Surgical oncology, Venice Italy, November 30-December 2, 2006. Abstract available online at http://www.sciencedirect.com.ezp-prod1.hul.harvard.edu/science?_ob=MImg&_imagekey=B6WF4-4MM7RM7-7N-1&_cdi=6784&_user=209690&_pii=S0748798306706503&_origin=browse&_zone=rslt_list_item&_coverDate=11%2F30%2F2006&_sk=999679999.8998&wchp=dGLbVzW-zSkzS&md5=9532dfbb86f060bb0bc3c495f9aceef2&ie=/sdarticle.pd (Accessed on January 25, 2011).
- Adem C, Reynolds C, Ingle JN, Nascimento AG. Primary breast sarcoma: clinicopathologic series from the Mayo Clinic and review of the literature. Br J Cancer 2004; 91:237.
- Ciatto S, Bonardi R, Cataliotti L, Cardona G. Sarcomas of the breast: a multicenter series of 70 cases. Neoplasma 1992; 39:375.
- Vorburger SA, Xing Y, Hunt KK, et al. Angiosarcoma of the breast. Cancer 2005; 104:2682.
- Donnell RM, Rosen PP, Lieberman PH, et al. Angiosarcoma and other vascular tumors of the breast. Am J Surg Pathol 1981; 5:629.
- Luini A, Gatti G, Diaz J, et al. Angiosarcoma of the breast: the experience of the European Institute of Oncology and a review of the literature. Breast Cancer Res Treat 2007; 105:81.
- Shet T, Malaviya A, Nadkarni M, et al. Primary angiosarcoma of the breast: observations in Asian Indian women. J Surg Oncol 2006; 94:368.
- Gladdy RA, Qin LX, Moraco N, et al. Do radiation-associated soft tissue sarcomas have the same prognosis as sporadic soft tissue sarcomas? J Clin Oncol 2010; 28:2064.
- Chen KT, Kirkegaard DD, Bocian JJ. Angiosarcoma of the breast. Cancer 1980; 46:368.
- Strobbe LJ, Peterse HL, van Tinteren H, et al. Angiosarcoma of the breast after conservation therapy for invasive cancer, the incidence and outcome. An unforseen sequela. Breast Cancer Res Treat 1998; 47:101.
- Marchal C, Weber B, de Lafontan B, et al. Nine breast angiosarcomas after conservative treatment for breast carcinoma: a survey from French comprehensive Cancer Centers. Int J Radiat Oncol Biol Phys 1999; 44:113.
- Sordillo PP, Chapman R, Hajdu SI, et al. Lymphangiosarcoma. Cancer 1981; 48:1674.
- Abraham JA, Hornicek FJ, Kaufman AM, et al. Treatment and outcome of 82 patients with angiosarcoma. Ann Surg Oncol 2007; 14:1953.
- Christensen L, Schiødt T, Blichert-Toft M, et al. Sarcomas of the breast: a clinico-pathological study of 67 patients with long term follow-up. Eur J Surg Oncol 1988; 14:241.
- Billings SD, McKenney JK, Folpe AL, et al. Cutaneous angiosarcoma following breast-conserving surgery and radiation: an analysis of 27 cases. Am J Surg Pathol 2004; 28:781.
- Seinen JM, Styring E, Verstappen V, et al. Radiation-associated angiosarcoma after breast cancer: high recurrence rate and poor survival despite surgical treatment with R0 resection. Ann Surg Oncol 2012; 19:2700.
- Patel SR, Zagars GK, Pisters PW. The follow-up of adult soft-tissue sarcomas. Semin Oncol 2003; 30:413.
- Lahat G, Dhuka AR, Lahat S, et al. Outcome of locally recurrent and metastatic angiosarcoma. Ann Surg Oncol 2009; 16:2502.
- des Guetz G, Piperno-Neumann S, Chapelier A, et al. Postirradiation sarcoma: clinicopathologic features and results of chemotherapy (abstract). Proc Am Soc Clin Oncol 2002; 21:404a.
- Tomasini C, Grassi M, Pippione M. Cutaneous angiosarcoma arising in an irradiated breast. Case report and review of the literature. Dermatology 2004; 209:208.
- Kuten A, Sapir D, Cohen Y, et al. Postirradiation soft tissue sarcoma occurring in breast cancer patients: report of seven cases and results of combination chemotherapy. J Surg Oncol 1985; 28:168.
- Waldmann V. Liposomal doxorubicin in the treatment of metastasized angiosarcoma. Br J Dermatol 2003; 148:606.
- Budd GT. Management of angiosarcoma. Curr Oncol Rep 2002; 4:515.
- Borden EC, Amato DA, Rosenbaum C, et al. Randomized comparison of three adriamycin regimens for metastatic soft tissue sarcomas. J Clin Oncol 1987; 5:840.
- Edmonson JH, Ryan LM, Blum RH, et al. Randomized comparison of doxorubicin alone versus ifosfamide plus doxorubicin or mitomycin, doxorubicin, and cisplatin against advanced soft tissue sarcomas. J Clin Oncol 1993; 11:1269.
- Baker LH, Frank J, Fine G, et al. Combination chemotherapy using adriamycin, DTIC, cyclophosphamide, and actinomycin D for advanced soft tissue sarcomas: a randomized comparative trial. A phase III, Southwest Oncology Group Study (7613). J Clin Oncol 1987; 5:851.
- Antman K, Crowley J, Balcerzak SP, et al. An intergroup phase III randomized study of doxorubicin and dacarbazine with or without ifosfamide and mesna in advanced soft tissue and bone sarcomas. J Clin Oncol 1993; 11:1276.
- Verweij J, Lee SM, Ruka W, et al. Randomized phase II study of docetaxel versus doxorubicin in first- and second-line chemotherapy for locally advanced or metastatic soft tissue sarcomas in adults: a study of the european organization for research and treatment of cancer soft tissue and bone sarcoma group. J Clin Oncol 2000; 18:2081.
- GENERAL PRINCIPLES
- Management of regional nodes
- ADJUVANT RT
- Primary breast sarcomas
- - Summary
- Radiotherapy-related breast sarcomas
- ADJUVANT CHEMOTHERAPY
- NEOADJUVANT THERAPY FOR LARGE LOCALLY ADVANCED INITIALLY UNRESECTABLE LESIONS
- POSTTREATMENT CANCER SURVEILLANCE
- TREATMENT OF RECURRENT OR METASTATIC DISEASE
- SUMMARY AND RECOMMENDATIONS