[Hemangiosarcoma after breast-conserving therapy of breast cancer: report of four cases with molecular genetic diagnosis and literature review]. / Hämangiosarkom nach brusterhaltender Therapie beim Mammakarzinom: vier Fallbeispiele mit molekulargenetischer Diagnostik und Literaturübersicht.

ABSTRACT

BACKGROUND: Hemangiosarcomas of the breast represent a rare disease of the breast mainly occurring as secondary neoplasias with a latency of 5-10 years after primary treatment of breast cancer and are associated with an unfavourable prognosis. Radiation therapy, which is integrated within the concept of breast conserving therapy ranks as the main risk factor. PATIENTS AND METHODS: In this report we describe the clinical course of 4 patients including their molecular genetic pattern and give a summary of the actual literature. RESULTS: Hemangiosarcomas occur as a secondary neoplasm with a latency of 5-10 years after primary treatment of breast cancer and have an unfavorable prognosis. A genetic predisposition is assumed, but we could not find a significant role of tumor suppressor genes BRCA1, BRCA2 or p53 in our patients. CONCLUSION: Due to limited data available for these tumors, recommendations for therapy include radical tumor resection achieving wide free margins and inconsistent regimens of chemo- and/or immunetherapy modalities. In the majority these are based on systemic therapy regimens for other cutaneous sarcomas, such as Kaposi's sarcoma. Efforts should be taken for a nation-wide systematic registration of all cases of post-irradiation hemangiosarcomas.