Primary Breast Sarcoma: A Case Report at Kuwait Cancer Center and Review of the Literature

Breast sarcomas can be classified as primary (de novo) or, more commonly, secondary to previous radiation therapy for breast cancer or lymphoma. Primary breast sarcoma (PBS) is particularly rare (around 0.1% of all primary breast cancers and <5% of all soft tissue sarcomas) and differs from malignant phyllodes tumors with a worse prognosis. Diagnosis is by triple assessment and ruling out all the differentials (most commonly malignant phyllodes and metaplastic carcinomas) using core biopsy, but sometimes complete surgical excision is needed to confirm the tissue diagnosis. Staging and management of PBS are different from that for breast carcinoma, and they follow the guidelines of soft tissue sarcoma of the trunk and extremities. The standard of care is surgical resection with adequate negative margins (>1 cm for all sarcomas and >3 cm in angiosarcoma and leiomyosarcoma). Axillary staging can be considered only if the core biopsy diagnosis is uncertain (complete excision advised by the pathologist) or in the presence of discordance between clinical/radiological findings and fine-needle cytology of axillary lymph nodes. The role of adjuvant therapy, either radiation or systemic chemotherapy, is highly controversial, but the National Comprehensive Cancer Network (NCCN) guidelines recommend considering adjuvant radiotherapy for cases with positive margins or higher stages. In addition, there is no solid consensus about the benefit of chemotherapy, but some articles reported using it in both adjuvant and neoadjuvant settings. In conclusion, the management should be on a case-by-case basis supported by multidisciplinary team (MDT) approach. The aim of this article is to present a case of PBS with a clinical presentation of rapidly growing locally advanced breast cancer in a 32-year-old woman in the State of Kuwait. Moreover, the authors are presenting an up-to-date review of the limited literature on PBS.