Department of Oncology, Shengjing Hospital of China Medical University, Shenyang, 110004, China.
BMC Cancer. 2023 Aug 28;23(1):801. doi: 10.1186/s12885-023-11279-2.
Giant breast malignant phyllodes tumor or sarcoma (GBPS) are rare entities with diameter larger than 10 cm and variously histological pleomorphisms. This disease poses a significant threat to the quality of life of individuals, and its prognosis remains unclear. This study aimed to explore the differential diagnosis, treatment, and prognosis of GBPS in a real-world retrospective cohort.
We collected GBPS (diameter > 10 cm, n = 10) and BPS (diameter ≤ 10 cm, n = 126) from patients diagnosed with sarcoma or malignant phyllodes tumor between 2008 and 2022. We analyzed clinical characteristics, histological status, treatment, and local recurrence using the Fisher's exact test between GBPS (diameter > 10 cm) and BPS (diameter ≤ 10 cm) cohort. We described overall survival (OS) and disease-free survival (DFS) using Kaplan-Meier curves and identified risk factors for local recurrence using logistic regression. The tumor size, age at diagnosis, and differential immunohistochemistry markers of breast sarcoma or phyllodes tumor to determine the prognosis of GBPS.
In our retrospective analysis of breast malignancies, we identified 10 cases of GBPS and 126 cases of BPS, corresponding to a GBPS prevalence of 0.17% (10/6000). The median age was 38.5 years (inter-quartile range, IQR: 28.25-48.5 years). During the follow-up of period (median: 80.5 months, IQR: 36.75-122 months), the local recurrence (LR) rate was 40% and 20.6%, respectively. Clinical characteristics of young age (HR:2.799, 95%CI -00.09276-0.017, p < 0.05) and cytological characteristics of marked stromal atypia (HR:0.88, 95% CI 0.39-1.40, p < 0.05) were risk factors for the poor prognosis of GBPS by COX regression model analysis. The Kaplan-Meier curves of GBPS 5-year disease-free survival (DFS) and overall survival (OS) were 31.5 months and 40 months, respectively, and were not associated with adjuvant radiation or chemotherapy.
We recommend mastectomy with a clear surgical margin as the preferred treatment for GBPS. Age and stromal atypia are significantly associated with recurrence. Adjuvant radiation therapy is advised; however, there was no improvement in overall survival. There is no consensus on the effectiveness of adjuvant chemotherapy and genetic methods, highlighting the need for further research into this aggressive tumor. We recommend a multidisciplinary approach involving a dedicated team for the management of GBPS.
直径大于 10cm 的巨大乳腺恶性叶状肿瘤或肉瘤（GBPS）是一种罕见的实体瘤，具有多种组织学多态性。这种疾病对个体的生活质量构成重大威胁，其预后仍不清楚。本研究旨在探讨真实世界回顾性队列中 GBPS 的鉴别诊断、治疗和预后。
我们收集了 2008 年至 2022 年间诊断为肉瘤或恶性叶状肿瘤的患者中直径大于 10cm 的 GBPS（n=10）和直径小于等于 10cm 的 BPS（n=126）。我们使用 Fisher 精确检验比较了 GBPS（直径大于 10cm）和 BPS（直径小于等于 10cm）队列之间的临床特征、组织学状态、治疗和局部复发情况。我们使用 Kaplan-Meier 曲线描述总生存（OS）和无病生存（DFS），并使用逻辑回归确定局部复发的风险因素。使用肿瘤大小、诊断时的年龄和乳腺肉瘤或叶状肿瘤的差异免疫组织化学标志物来确定 GBPS 的预后。
在我们对乳腺恶性肿瘤的回顾性分析中，我们确定了 10 例 GBPS 和 126 例 BPS，GBPS 的患病率为 0.17%（10/6000）。中位年龄为 38.5 岁（四分位距，IQR：28.25-48.5 岁）。在中位随访期 80.5 个月（IQR：36.75-122 个月）期间，局部复发（LR）率分别为 40%和 20.6%。通过 COX 回归模型分析，年轻的临床特征（HR：2.799，95%CI-00.09276-0.017，p<0.05）和明显的间质细胞异型性的细胞学特征（HR：0.88，95%CI 0.39-1.40，p<0.05）是 GBPS 预后不良的危险因素。GBPS 的 5 年无病生存率（DFS）和总生存率（OS）的 Kaplan-Meier 曲线分别为 31.5 个月和 40 个月，与辅助放疗或化疗无关。
我们建议行明确切缘的乳房切除术作为 GBPS 的首选治疗方法。年龄和间质细胞异型性与复发显著相关。建议行辅助放疗；然而，总生存率并没有改善。辅助化疗和基因方法的有效性尚无共识，这突出表明需要对这种侵袭性肿瘤进行进一步研究。我们建议采用多学科方法，由专门的团队来管理 GBPS。
