Clinical perspectives and outcomes of the giant breast phyllodes tumor and sarcoma: a real-world retrospective study.
Clinical perspectives and outcomes of the giant breast phyllodes tumor and sarcoma: a real-world retrospective study.
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DOI：
10.1186/s12885-023-11279-2
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发表时间：
2023-08-28
期刊：
影响因子：
3.8
通讯作者：
Han, Ye
中科院分区：
文献类型：
--
作者：
Liu, Naiquan;Kang, Ye;Qu, Ningxin;Kong, Chenhui;Han, Ye
摘要巨大乳房恶性叶状肿瘤或肉瘤是一种罕见的肿瘤，直径大于10公分，且具有多种组织学上的多形性。这种疾病对个人的生活质量构成重大威胁，其预后仍不清楚。本研究旨在通过一个真实世界的回顾性队列研究来探讨GBPS的鉴别诊断、治疗和预后。我们收集了2008年至2022年诊断为肉瘤或恶性叶状肿瘤的患者的GBPS（直径> 10 cm，n = 10）和BPS（直径≤ 10 cm，n = 126）。我们使用Fisher精确检验分析了GBPS（直径> 10 cm）和BPS（直径≤ 10 cm）队列的临床特征、组织学状态、治疗和局部复发。我们使用Kaplan-Meier曲线描述了总生存期（OS）和无病生存期（DFS），并使用logistic回归确定了局部复发的危险因素。肿瘤大小、诊断时的年龄、乳腺肉瘤或叶状肿瘤的鉴别免疫组化标志物可确定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，相当于GBPS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）。随访期（中位数：80.5个月，IQR：36.75 ~ 122个月），局部复发率（LR）分别为40%和20.6%。考克斯回归模型分析显示，年龄小（HR：2.799，95%CI-00.09276 - 0.017，p < 0.05）和间质细胞学特征明显（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年无病生存期（DFS）和总生存期（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。我们建议将具有清晰手术边缘的乳房切除术作为GBPS的首选治疗方法。年龄和间质增生与复发显著相关。建议进行辅助放射治疗;然而，总体生存率没有改善。对于辅助化疗和遗传方法的有效性还没有达成共识，这突出了对这种侵袭性肿瘤进行进一步研究的必要性。我们建议采用多学科方法，包括一个专门的团队来管理GBPS。在线版本包含补充材料，可通过10.1186/s12885-023-11279-2获得。
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. The online version contains supplementary material available at 10.1186/s12885-023-11279-2.
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DOI：
10.1016/j.ijrobp.2007.06.059
发表时间：
2008-02-01
影响因子：
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