Localized Angiosarcoma, Not One Disease: A Retrospective Single-Center Study on Prognosis Depending on the Primary Site and Etiology.

Q2 Medicine

Sarcoma Pub Date : 2021-09-10 eCollection Date: 2021-01-01 DOI:10.1155/2021/9960085

Inna Schott, Sven-Thorsten Liffers, Farhad Farzaliyev, Johanna Falkenhorst, Hans-Ulrich Steinau, Jürgen-Walter Treckmann, Lars Erik Podleska, Christoph Pöttgen, Hans-Ulrich Schildhaus, Marit Ahrens, Uta Dirksen, Fatma-Zehra Murat, Jens T Siveke, Sebastian Bauer, Rainer Hamacher

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引用次数: 0

Abstract

Background: Angiosarcomas are rare and heterogeneous tumors with poor prognosis. The clinical subtypes are classified depending on the primary site and etiology.

Methods: We conducted a retrospective, monocentric study of 136 patients with localized AS between May 1985 and November 2018. Overall survival (OS), local recurrence-free survival (LRFS), and metastasis-free survival (MFS) were estimated using the Kaplan-Meier method. To identify prognostic factors, univariate and multivariate analyses were performed based on Cox regressions.

Results: The median age was 67 years (19-72.8 years). Primary sites were cutaneous (27.2%), breast (38.2%), and deep soft tissue (34.6%). The majority was primary angiosarcomas (55.9%) followed by postradiation (40.4%) and chronic lymphedema angiosarcomas (2.9%). Prognosis significantly differed depending on the primary site and etiology. Shortest median OS and MFS were observed in deep soft tissue angiosarcomas, whereas cutaneous angiosarcomas, angiosarcomas of the breast, and radiation-associated angiosarcomas displayed worse median LRFS. Univariate analyses showed better OS for tumor size <10 cm (p = 0.009), negative surgical margins (p = 0.021), and negative lymph node status (p = 0.007). LRFS and MFS were longer for tumor size <10 cm (p = 0.012 and p = 0.013). In multivariate analyses, age <70 years was the only independent positive prognostic factor for OS in all subgroups. For LRFS, secondary AS of the breast was a negative prognostic factor (HR: 2.35; p = 0.035).

Conclusions: Different behaviors and prognoses depending on the primary site and etiology should be considered for the treatment of this heterogeneous disease. In cutaneous angiosarcomas of the head/neck and postradiation angiosarcomas of the breast, local recurrence seems to have a crucial impact on OS. Therefore, improved local therapies and local tumor staging may have to be implemented. However, in deep soft tissue angiosarcomas, distant recurrence seems to have a major influence on prognosis, which indicates a benefit of additional perioperative chemotherapy.

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局部血管肉瘤，不是一种疾病：根据原发部位和病因判断预后的单中心回顾性研究

背景：血管肉瘤是一种罕见的异质性肿瘤，预后较差。临床亚型根据原发部位和病因进行分类：我们对1985年5月至2018年11月期间的136例局部AS患者进行了回顾性、单中心研究。采用 Kaplan-Meier 法估算了总生存期（OS）、无局部复发生存期（LRFS）和无转移生存期（MFS）。为了确定预后因素，根据 Cox 回归进行了单变量和多变量分析：中位年龄为 67 岁（19-72.8 岁）。原发部位为皮肤（27.2%）、乳腺（38.2%）和深部软组织（34.6%）。大多数是原发性血管肉瘤（55.9%），其次是放疗后血管肉瘤（40.4%）和慢性淋巴水肿血管肉瘤（2.9%）。根据原发部位和病因的不同，预后也明显不同。深部软组织血管肉瘤的中位OS和MFS最短，而皮肤血管肉瘤、乳腺血管肉瘤和放射相关血管肉瘤的中位LRFS较差。单变量分析显示，肿瘤大小（P = 0.009）、手术切缘阴性（P = 0.021）和淋巴结状态阴性（P = 0.007）的患者OS较好。肿瘤大小（p = 0.012 和 p = 0.013）的 LRFS 和 MFS 更长。在多变量分析中，年龄 p = 0.035）：结论：在治疗这种异质性疾病时，应根据原发部位和病因考虑不同的行为和预后。在头颈部皮肤血管肉瘤和乳腺放疗后血管肉瘤中，局部复发似乎对OS有着至关重要的影响。因此，必须改进局部疗法和局部肿瘤分期。然而，在深部软组织血管肉瘤中，远处复发似乎对预后有重大影响，这表明围手术期额外化疗的益处。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Sarcoma Medicine-Radiology, Nuclear Medicine and Imaging

CiteScore

5.00

自引率

0.00%

发文量

审稿时长

14 weeks

期刊介绍： Sarcoma is dedicated to publishing papers covering all aspects of connective tissue oncology research. It brings together work from scientists and clinicians carrying out a broad range of research in this field, including the basic sciences, molecular biology and pathology and the clinical sciences of epidemiology, surgery, radiotherapy and chemotherapy. High-quality papers concerning the entire range of bone and soft tissue sarcomas in both adults and children, including Kaposi"s sarcoma, are published as well as preclinical and animal studies. This journal provides a central forum for the description of advances in diagnosis, assessment and treatment of this rarely seen, but often mismanaged, group of patients.