What Is the Impact of Multimodal Treatment in Patients with Leiomyosarcoma of Bone? A Multicenter Study of 35 Patients with an Ultra-Rare Tumor Entity

Author:

Niethard Maya12ORCID,Knebel Carolin3ORCID,Leithner Andreas4ORCID,Tunn Per-Ulf1,Schoon Janosch2ORCID,Reichardt Peter5,Pogkas Athanasios6ORCID,Szkandera Joanna7,Pink Daniel8ORCID,Andreou Dimosthenis49ORCID

Affiliation:

1. Department of Orthopedic Oncology, Sarcoma Center, Helios Klinikum Berlin-Buch, 13125 Berlin, Germany

2. Center for Orthopedics, Trauma Surgery and Rehabilitation Medicine, University Medicine Greifswald, Ferdinand-Sauerbruch-Straße, 17475 Greifswald, Germany

3. Department of Orthopedics and Sports Orthopedics, Klinikum Rechts der Isar, Technical University of Munich, 81675 Munich, Germany

4. Department of Orthopedics and Trauma, Medical University of Graz, 8036 Graz, Austria

5. Department of Oncology, Sarcoma Center, Helios Klinikum Berlin-Buch, 13125 Berlin, Germany

6. Department of Hematology, Oncology and Palliative Medicine, Vivantes Klinikum Berlin Neukölln, Rudower Straße 48, 12351 Berlin, Germany

7. Division of Clinical Oncology, Department of Internal Medicine, Medical University of Graz, 8036 Graz, Austria

8. Department of Oncology, Sarcoma Center Berlin-Brandenburg, Helios Klinikum, 15526 Bad Saarow, Germany

9. Department of General Orthopedics and Tumor Orthopedics, Münster University Hospital, 48149 Münster, Germany

Abstract

Primary leiomyosarcoma of bone (LMSoB) is extremely rare, comprising only <0.7% of primary malignant bone tumors, and is therefore considered an ultra-rare tumor entity. There is currently no consensus as to whether therapeutic strategies should be based on the biological characteristics of soft tissue leiomyosarcoma or on primary tumor localization in the bone. The use of perioperative chemotherapy and its effectiveness in this rare tumor entity remains unclear. We aimed to evaluate the impact of different treatment approaches in a multicenter setting with a total of 35 patients included. The 5-year overall survival (OS) was 74%. Patients with localized disease undergoing surgery had a significantly higher 5-year OS compared to patients who did not undergo surgical treatment (82% vs. 0%, p = 0.0015). Axial tumor localization was associated with worse event-free survival (EFS) probability (p < 0.001) and OS (p = 0.0082). A high proportion of our patients developed secondary metastases. Furthermore, the perioperative chemotherapy protocols applied to our patients were not associated with an improved EFS or OS. Therefore, the benefit of perioperative chemotherapy in LMSoB needs to be further investigated, and the choice of agents still needs to be clarified.

Publisher

MDPI AG

同舟云学术

1.学者识别学者识别

2.学术分析学术分析

3.人才评估人才评估

"同舟云学术"是以全球学者为主线,采集、加工和组织学术论文而形成的新型学术文献查询和分析系统,可以对全球学者进行文献检索和人才价值评估。用户可以通过关注某些学科领域的顶尖人物而持续追踪该领域的学科进展和研究前沿。经过近期的数据扩容,当前同舟云学术共收录了国内外主流学术期刊6万余种,收集的期刊论文及会议论文总量共计约1.5亿篇,并以每天添加12000余篇中外论文的速度递增。我们也可以为用户提供个性化、定制化的学者数据。欢迎来电咨询!咨询电话:010-8811{复制后删除}0370

www.globalauthorid.com

TOP

Copyright © 2019-2024 北京同舟云网络信息技术有限公司
京公网安备11010802033243号  京ICP备18003416号-3