Soft tissue sarcomas are very rare malignancies of the breast, often due to previous breast radiotherapy.
Pathology
In contrast to other sites, fibrosarcoma and angiosarcoma are equally as prevelant as the usually more common pleomorphic sarcoma, each making up about 1/4 of diagnoses. Angiosarcomas are typically secondary to treatment (< 0.5% risk) and have a more aggressive course locally and distantly.
Diagnosis
Patients present with an enlarging mass within the breast. It can be painful or ulcerate in large cases. Angiosarcoma is the exception as it often locally infiltrates the skin, leading to an area of ulceration or discolouration. Biopsy may be difficult to interpret.
Staging
Staging is as for soft tissue sarcoma at other sites, based on histological grade, depth (superficial/deep), size < or > than 5 cm, lymph nodes and distant metastases.
Management
Surgery is the mainstay of treatment and is used for all potentially curative cases.
Adjuvant radiotherapy has a role in primary breast sarcomas, which are treated similarly to soft tissue sarcoma at other sites. There is a paucity of evidence for this treatment.
Angiosarcoma secondary to radiotherapy presents an interesting dilemma. With surgery alone, recurrences occur in > 50% of patients. Adding radiotherapy reduces this risk but runs the competing risk of late radiotherapy complications in a doubly treated area. Most series evaluating radiotherapy have used hyperfractionated regimens.
Chemotherapy has a limited role similar to soft tissue sarcoma at other sites. There is conflicting evidence for benefit and this should be assessed on a case by case basis; ifosfamide and doxorubicin have the most positive impact in most reviews.
Outcomes
Recurrences occur in 50% of patients and most of these patients will die of their disease. Rates are higher for angiosarcoma of equal stage.
