Ramalho Inês, Campos Sara, Rebelo Teresa, Figueiredo Dias Margarida
Gynecology A Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.
Case Rep Oncol. 2016 Nov 25;9(3):796-801. doi: 10.1159/000452946. eCollection 2016 Sep-Dec.
Primary breast sarcoma, arising from connective tissue within the breast, is extremely rare, accounting for less than 1% of all primary breast malignancies and no more than 5% of all sarcomas. The rarity of this pathology limits most studies to case reports and small retrospective studies, which has led to a lack of consensus on the clinical management. We report a clinical case of a 52-year-old woman, perimenopausal, previously healthy, with regular breast surveillance, who presented with a large (>20 cm) and rapidly expanding hypervascularized tumor of the left breast developed over 10 days, with a very thin preulcerative skin over the last 4 days. There was no systemic dissemination. The patient was submitted to total mastectomy and excision of axillary adenopathy. The tumor was diagnosed histologically as malignant phyllodes tumor associated with areas of high-grade sarcoma. Due to rapid growth and aggressive histological characteristics, adjuvant chemotherapy and radiotherapy were performed. There is a lot of evidence that tumors larger than 5 cm are associated with a poor prognosis. Despite the poor prognosis associated with this aggressive entity, the patient had no recurrence during 5 years of follow-up. We review the relevant literature about primary breast sarcomas.
原发性乳腺肉瘤起源于乳腺内的结缔组织，极为罕见，占所有原发性乳腺恶性肿瘤的比例不到1%，占所有肉瘤的比例不超过5%。这种病理情况的罕见性使得大多数研究局限于病例报告和小型回顾性研究，这导致在临床管理方面缺乏共识。我们报告一例临床病例，患者为52岁围绝经期女性，既往健康，有定期乳腺检查，其左乳出现一个大（>20 cm）且迅速增大的高血管化肿瘤，在10天内形成，在过去4天里肿瘤表面皮肤极薄且有溃疡前表现。无全身转移。患者接受了全乳切除术及腋窝淋巴结清扫术。肿瘤经组织学诊断为恶性叶状肿瘤合并高级别肉瘤区域。鉴于肿瘤生长迅速且组织学特征侵袭性强，进行了辅助化疗和放疗。有大量证据表明，肿瘤大于5 cm与预后不良相关。尽管这种侵袭性疾病预后不佳，但该患者在5年随访期间未复发。我们回顾了关于原发性乳腺肉瘤的相关文献。
