Primary breast sarcoma: clinicopathologic series from the Mayo Clinic and review of the literature

Abstract

Primary sarcomas of the breast are extremely rare, with less than 0.1% of all malignant tumours of the breast. Mayo Clinic Surgical Pathology database was searched for all breast sarcoma from 1910 to 2000. Pathology reports and slides were reviewed and tumour types were determined. Metaplastic carcinomas and phyllodes tumours were excluded. There were 25 women ranging in age 24 -81 years (mean 45 years). All but one patient presented with a palpable lump. Mastectomy was performed in 19 patients and lumpectomy in five patients. Histopathological diagnoses were fibrosarcoma (six), angiosarcoma (six), pleomorphic sarcoma (six), leiomyosarcoma (two), myxofibrosarcoma (three), hemangiopericytoma (one) and osteosarcoma (one). Tumour size ranged from 0.3 to 12 cm (mean 5.7). Low-grade lesions were observed in 10 cases and high-grade in 15. Overall, mean follow-up was 10.5 years. Local recurrence was observed in 11 patients and ranged from 2 to 36 months (mean 15 m), while distant metastasis was observed in 10 patients (40%) affecting lungs, bones, liver, spleen, and skin. Of the 25 patients, 12 have died of disease and six of other causes. Five-year overall (OS) and cause-specific survival (CSS) were 66 and 70%, respectively. OS and DFS at 5 years were 91% for tumours p5 cm and 50% for tumours 45 cm. Tumour size was significantly associated with OS (risk ratio ¼ 1.3 per 1 cm increase; 95% CI, 1.02 -1.7; P ¼ 0.036). There was no significant difference in OS or CSS between low-and high-grade lesions. In this series, tumour size was a more valuable prognostic factor than tumour grade. Primary sarcomas of the breast are rare, malignant tumours arising from the mesenchymal tissue of the mammary gland (Oberman, 1965;Barnes and Pietruszka, 1977;Callery et al, 1985), with an approximate incidence of 17 new cases per million women (Moore and Kinne, 1996). At the Mayo Clinic, 27 881 malignant breast tumours were seen between 1940 and 1999 (C Adem, personal unpublished data) and 18 breast sarcomas were diagnosed accounting for 0.0006% of breast malignancies.Breast sarcomas should be distinguished from metaplastic carcinomas (Adem et al, 2002). When facing a spindle cell neoplasm in an epithelial organ such as the breast one should be careful in rendering the diagnosis of sarcoma. In this setting, immunohistochemistry using the right antibodies is of major input. Berg et al defined stromal sarcomas of the breast in 1962 as a group of mesenchymal malignant tumours with fibrous, myxoid and adipose components, excluding malignant cystosarcoma phyllodes, lymphomas and angiosarcomas (Berg et al, 1962). However, series in the literature have included many different entities under the rubric of sarcomas such as cystosarcoma phyllodes, lymphosarcoma and carcinosarcoma (Botham et al, 1958;Donegan, 1967;Fawcett, 1967;Kennedy and Biggart, 1967;Rissanen and Holsti, 1968;Gogas et al, 1976;Ludgate et al, 1977;Khanna et al, 1981;Christensen et al, 1988;Terrier et al, 1989;Pitts et al, 1991;Ciatto et al, 1992;Luna Vega et al, 1992;McGregor et al,...

Treatment for breast sarcoma: A large, single-centre series

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Osteosarcoma Breast - A Case Report

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Primary Angiosarcoma of the Breast—Series of 11 Consecutive Cases—A Single-Centre Experience

“…Complete excision is the key treatment modality, with preference given to mastectomy 5 . Such a procedure was performed in all of our patients.…”

“…It is most commonly seen in younger patients, women in their thirties and forties 4 . Clinically, women with pas usually present with swelling, a sensation of fullness, and exponential growth within the breast 5,6 . The prognosis for pas is generally poor, and the diagnosis may be delayed because of possible unusual clinical presentation and its rarity.…”