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辐射诱导性乳腺肉瘤的临床与分子学研究进展:这种侵袭性疾病的有效治疗是否有新的希望?

Clinical and Molecular Insights of Radiation-Induced Breast Sarcomas: Is There Hope on the Horizon for Effective Treatment of This Aggressive Disease?

机构信息

First Department of Pathology, Medical School, National and Kapodistrian University of Athens, 75 Mikras Asias Street, 11527 Athens, Greece.

Oncology Unit, 2nd Department of Medicine, Medical School, National and Kapodistrian University of Athens, Hippocratio General Hospital of Athens, V. Sofias 114, 11527 Athens, Greece.

出版信息

Int J Mol Sci. 2022 Apr 8;23(8):4125. doi: 10.3390/ijms23084125.

Abstract

Radiation-induced breast sarcomas (RIBS) are rare entities representing <1% of all primary breast malignancies, limiting most reports to small retrospective case series. They constitute a heterogeneous group of neoplasms, with high-grade angiosarcoma being the most common subtype. Other sarcoma histotypes, such as undifferentiated pleomorphic sarcoma and leiomyosarcoma, can also be identified. Radiation-induced breast angiosarcoma (RIBA) has an incidence of approximately 0.1% after breast-conserving therapy and arises mainly from the dermis of the irradiated breast. MYC gene amplification is highly indicative of secondary breast angiosarcomas. Their clinical presentation often mimics benign port-radiation lesions, leading to a delay in diagnosis and a lost window of opportunity for cure. Surgery with negative margins is the mainstay of treatment of localized RIBS. In the case of angiosarcoma, technical difficulties, including multifocality, infiltrative margins, and difficulty in assessing tumor margins, render surgical treatment quite challenging. A limited number of studies showed that adjuvant radiation therapy reduces local recurrences; therefore, it is proposed by many groups for large, high-grade tumors. Chemotherapy has been evaluated retrospectively in a small subset of patients, with some evidence supporting its use in angiosarcoma patients. Approximately half of patients with RIBA will show local recurrence. In the advanced setting, different therapeutic options are discussed in the review, including chemotherapy, antiangiogenic therapy, and immunotherapy, whereas the need for further research on molecular therapeutic targets is pointed out.

摘要

放射性诱导的乳腺肉瘤(RIBS)是罕见的实体瘤,占所有原发性乳腺恶性肿瘤的<1%,这使得大多数报告仅限于小型回顾性病例系列。它们是一组异质性肿瘤,高级别血管肉瘤是最常见的亚型。其他肉瘤组织类型,如未分化多形性肉瘤和平滑肌肉瘤,也可以被识别。保乳治疗后,放射性诱导的乳腺血管肉瘤(RIBA)的发病率约为 0.1%,主要发生在受照射乳腺的真皮层。MYC 基因扩增高度提示继发性乳腺血管肉瘤。它们的临床表现常模仿良性放射性损伤病变,导致诊断延迟,错失治愈机会。手术切除并保证切缘阴性是局限性 RIBS 的主要治疗方法。在血管肉瘤的情况下,由于存在多发病灶、浸润性切缘以及评估肿瘤切缘的困难等技术问题,手术治疗极具挑战性。少数研究表明,辅助放疗可降低局部复发率;因此,许多研究组建议对大体积、高级别的肿瘤进行辅助放疗。一些回顾性研究评估了化疗在小部分患者中的应用,有证据表明其对血管肉瘤患者有效。约一半的 RIBA 患者会出现局部复发。在晚期,本综述讨论了不同的治疗选择,包括化疗、抗血管生成治疗和免疫治疗,同时指出需要进一步研究分子治疗靶点。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8818/9029574/8c414dd004e4/ijms-23-04125-g001.jpg

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