Nonepithelial Malignancies of the Breast

Breast sarcomas are rare clinical entities. The symptoms are similar to other breast malignancies, and diagnosis depends on conventional triple assessment. However, their biology and prognosis are closer to those of soft tissue sarcomas than to invasive breast cancers. Surgical excision with clear margins is the primary treatment for localized tumors. Lymph node sampling and dissection is not recommended. Adjuvant or neoadjuvant therapy should be considered for high-risk patients. Postoperative radiotherapy shows a small survival advantage, and response rates for systemic therapies are poor. Angiosarcomas are the most common sarcomas of the breast. They can be associated with lymphedema or irradiation. Surgery is the primary treatment, and wide negative margins are essential for a long-term cure. The potential for local recurrence and distant metastasis are high. Leiomyosarcoma, liposarcoma, rhabdomyosarcoma, and fibrosarcoma are the other rare sarcoma subtypes in the breast. Primary breast lymphoma is a rare entity that arises from the periductal and perilobular lymphatic tissue and intramammary lymph nodes. The breast is the first major site of manifestations without any evidence of concurrent systemic disease. A painless, mobile, rapidly enlarging mass is a common clinical presentation. Primary breast lymphomas are sensitive to chemotherapy and radiotherapy. Surgery is limited to biopsy. The clinical stage and histological subtype are the main prognostic factors. Metastatic involvement of the breast most often originates from the contralateral site. The most common malignancy of the body that metastasizes to the breast is malignant melanoma. Hematological malignancies such as leukemia and lymphoma also frequently occur. Comprehensive screening is necessary to identify the origin of the primary tumor.