Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
SHR Neuro Krebs Kardio Lipid Stoffw Microb
Niethard, M; Knebel, C; Leithner, A; Tunn, PU; Schoon, J; Reichardt, P; Pogkas, A; Szkandera, J; Pink, D; Andreou, D.
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.
Cancers (Basel). 2024; 16(9): 1633
Doi: 10.3390/cancers16091633
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- Co-Autor*innen der Med Uni Graz
- Andreou Dimosthenis
- Leithner Andreas
- Szkandera Joanna
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- 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.
- Find related publications in this database (Keywords)
- leiomyosarcoma of bone
- bone sarcoma
- systemic chemotherapy
- surgical treatment
- patient outcome
- rare primary malignant bone sarcoma (RPMBS)
- ultra-rare sarcoma
- multimodality treatment
- non-osteosarcoma malignant bone tumors