Advances in Diagnosis and Treatment for Bone and Soft Tissue Sarcoma

A special issue of Cancers (ISSN 2072-6694). This special issue belongs to the section "Cancer Therapy".

Deadline for manuscript submissions: closed (31 January 2024) | Viewed by 5895

Special Issue Editor


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Guest Editor
Department of Musculoskeletal Oncology and Orthopaedic Surgery, Tochigi Cancer Center, Tochigi 320-0834, Japan
Interests: sarcoma; rare cancer; biobank; patient derived cancer model
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Sarcoma is one of the rare cancers. Historically, cancer treatment began with surgery and evolved into radiation therapy and chemotherapy. The development of these multimodal therapies has significantly improved the outcome of cancer treatments, including sarcomas.

At present, the development of molecular-targeted therapeutic agents and immune checkpoint inhibitors is expected to further improve the treatment results for many carcinomas.

Unfortunately, sarcomas have not fully benefited from these new therapeutic agents including molecular-targeted therapies and immune checkpoint inhibitors. This is thought to be due to the social problem that the market for novel treatment development for sarcoma is very small and the development of new drugs is not progressing because the sarcoma is one of the rare cancers, and the peculiarity of the sarcoma itself, which is different from the carcinoma. Due to these problems, sarcoma treatment has not been developed for decades.

In this Special issue, I would like to mention the current status of diagnosis and treatments for sarcoma, and the possibilities of developing novel diagnosis methods and treatments in the future.

Prof. Dr. Kazutaka Kikuta
Guest Editor

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Keywords

  • sarcoma
  • rare cancer
  • diagnosis
  • treatment
  • molecular-targeted therapeutic agents
  • immune checkpoint inhibitors

Published Papers (5 papers)

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Research

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9 pages, 1407 KiB  
Article
The Combined Use of Inflammation Markers, Modified Glasgow Prognostic Score, and Sarculator Nomogram in Extremity Soft Tissue Sarcoma: A Multicenter Observational Study
by Tomoki Nakamura, Satoshi Takenaka, Hidetatsu Outani, Tomohito Hagi, Hironari Tamiya, Yoshinori Imura, Kunihiro Asanuma and Akihiro Sudo
Cancers 2024, 16(5), 1077; https://doi.org/10.3390/cancers16051077 - 06 Mar 2024
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Abstract
Background: Sarculator is a validated nomogram designed to predict overall survival (OS) in extremity soft tissue sarcoma (STS). Inflammation plays a critical role in cancer development and progression. There were no reports which investigated the relationship between Sarculator and inflammation. Methods: A total [...] Read more.
Background: Sarculator is a validated nomogram designed to predict overall survival (OS) in extremity soft tissue sarcoma (STS). Inflammation plays a critical role in cancer development and progression. There were no reports which investigated the relationship between Sarculator and inflammation. Methods: A total of 217 patients with extremity STS were included. The Sarculator-predicted 10-year probability of OS (pr-OS) was stratified into two subgroups: lower risk (10-year pr-OS ≥ 60%) and higher risk (10-year pr-OS < 60%). The modified Glasgow prognostic score (mGPS) varied from 0 to 2. Results: Out of the 217 patients, 67 were classified as higher risk, while 150 were lower risk. A total of 181 patients had an mGPS of 0, and 36 had a score of 1 or 2. The 5-year OS was 83.3%. When patients were divided into two groups according to the 10-year pr-OS, those with a higher risk had poorer OS than those with a lower risk. Among the patients with a higher risk, those with an mGPS of 1 or 2 had poorer OS compared to those with a score of 0. Conclusions: The mGPS could potentially play an important role in identifying patients who are at high risk of death and metastasis in the higher-risk group on the Sarculator. Full article
(This article belongs to the Special Issue Advances in Diagnosis and Treatment for Bone and Soft Tissue Sarcoma)
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14 pages, 1721 KiB  
Article
Refusal of Adjuvant Therapies and Its Impact on Local Control and Survival in Patients with Bone and Soft Tissue Sarcomas of the Extremities and Trunk
by Franziska Mentrup, Alexander Klein, Lars Hartwin Lindner, Silke Nachbichler, Boris Michael Holzapfel, Markus Albertsmeier, Thomas Knösel and Hans Roland Dürr
Cancers 2024, 16(2), 239; https://doi.org/10.3390/cancers16020239 - 05 Jan 2024
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Abstract
Background: In soft tissue or bone sarcomas, multimodal therapeutic concepts represent the standard of care. Some patients reject the therapeutic recommendations due to several reasons. The aim of this study was to assess the impact of that rejection on both prognosis and local [...] Read more.
Background: In soft tissue or bone sarcomas, multimodal therapeutic concepts represent the standard of care. Some patients reject the therapeutic recommendations due to several reasons. The aim of this study was to assess the impact of that rejection on both prognosis and local recurrence. Methods: Between 2012 and 2019, a total of 828 sarcoma patients were surgically treated. Chemotherapy was scheduled as a neoadjuvant, and adjuvant multi-agent therapy was performed following recommendations from an interdisciplinary tumor board. Radiotherapy, if deemed appropriate, was administered either in a neoadjuvant or an adjuvant manner. The recommended type of therapy, patient compliance, and the reasons for refusal were documented. Follow-ups included local recurrences, diagnosis of metastatic disease, and patient mortality. Results: Radiotherapy was recommended in 407 (49%) patients. A total of 40 (10%) individuals did not receive radiation. A reduction in overall survival and local recurrence-free survival was evident in those patients who declined radiotherapy. Chemotherapy was advised for 334 (40%) patients, 250 (75%) of whom did receive all recommended cycles. A total of 25 (7%) individuals did receive a partial course while 59 (18%) did not receive any recommended chemotherapy. Overall survival and local recurrence-free survival were reduced in patients refusing chemotherapy. Overall survival was worst for the group of patients who received no chemotherapy due to medical reasons. Refusing chemotherapy for non-medical reasons was seen in 8.8% of patients, and refusal of radiotherapy for non-medical reasons was seen in 4.7% of patients. Conclusions: Divergence from the advised treatment modalities significantly impacted overall survival and local recurrence-free survival across both treatment modalities. There is an imperative need for enhanced physician-patient communication. Reducing treatment times, as achieved with hypofractionated radiotherapy and with therapy in a high-volume sarcoma center, might also have a positive effect on complying with the treatment recommendations. Full article
(This article belongs to the Special Issue Advances in Diagnosis and Treatment for Bone and Soft Tissue Sarcoma)
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13 pages, 40681 KiB  
Article
Incidence and Risk Factors for Extremity Osteoradionecrosis after Limb-Sparing Surgery and Adjuvant Radiotherapy
by Yun-Jui Lu, Chun-Chieh Chen, Shih-Heng Chen, Cheng-Hung Lin, Yu-Te Lin, Chih-Hung Lin and Chung-Chen Hsu
Cancers 2023, 15(8), 2339; https://doi.org/10.3390/cancers15082339 - 17 Apr 2023
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Abstract
Osteoradionecrosis (ORN) is a major complication after radiotherapy. Most studies on ORN have focused on patients with mandibular lesions, with few studies including patients with extremity soft tissue sarcoma (STS). We included 198 patients with extremity STS who underwent limb-sparing surgery and adjuvant [...] Read more.
Osteoradionecrosis (ORN) is a major complication after radiotherapy. Most studies on ORN have focused on patients with mandibular lesions, with few studies including patients with extremity soft tissue sarcoma (STS). We included 198 patients with extremity STS who underwent limb-sparing surgery and adjuvant radiotherapy between 2004 and 2017. The incidence rate of extremity ORN was 3.5% (7/198), with most lesions (6/7) located in the lower extremities. The mean follow-up time was 62 months. Clinical presentations included chronic ulcers, soft tissue necrosis, sinus discharge, bone nonunion, and pathological fractures. Compared with the non-ORN group, the ORN group had a significantly higher total radiation dose (68 Gy vs. 64 Gy, p = 0.048) and greater use of intraoperative periosteal stripping (p = 0.008). Repeat surgeries and subsequent soft tissue reconstruction or limb amputation were performed as treatments. The risk and management of ORN in patients with extremity STS was ignored previously. Because the disease is complex and affects both clinicians and patients, careful surveillance should be undertaken. Full article
(This article belongs to the Special Issue Advances in Diagnosis and Treatment for Bone and Soft Tissue Sarcoma)
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Review

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18 pages, 1965 KiB  
Review
Modeling Myxofibrosarcoma: Where Do We Stand and What Is Missing?
by Enrico Lucarelli, Alessandro De Vita, Chiara Bellotti, Tommaso Frisoni, Silvia Vanni, Ania Naila Guerrieri, Micaela Pannella, Laura Mercatali, Marco Gambarotti, Serena Duchi, Giacomo Miserocchi, Margherita Maioli, Chiara Liverani and Toni Ibrahim
Cancers 2023, 15(21), 5132; https://doi.org/10.3390/cancers15215132 - 25 Oct 2023
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Abstract
Myxofibrosarcoma (MFS) is a malignant soft tissue sarcoma (STS) that originates in the body’s connective tissues. It is characterized by the presence of myxoid (gel-like) and fibrous components and typically affects patients after the fifth decade of life. Considering the ongoing trend of [...] Read more.
Myxofibrosarcoma (MFS) is a malignant soft tissue sarcoma (STS) that originates in the body’s connective tissues. It is characterized by the presence of myxoid (gel-like) and fibrous components and typically affects patients after the fifth decade of life. Considering the ongoing trend of increasing lifespans across many nations, MFS is likely to become the most common musculoskeletal sarcoma in the future. Although MFS patients have a lower risk of developing distant metastases compared with other STS cases, MFS is characterized by a high frequency of local recurrence. Notably, in 40–60% of the patients where the tumor recurs, it does so multiple times. Consequently, patients may undergo multiple local surgeries, removing the risk of potential amputation. Furthermore, because the tumor relapses generally have a higher grade, they exhibit a decreased response to radio and chemotherapy and an increased tendency to form metastases. Thus, a better understanding of MFS is required, and improved therapeutic options must be developed. Historically, preclinical models for other types of tumors have been instrumental in obtaining a better understanding of tumor development and in testing new therapeutic approaches. However, few MFS models are currently available. In this review, we will describe the MFS models available and will provide insights into the advantages and constraints of each model. Full article
(This article belongs to the Special Issue Advances in Diagnosis and Treatment for Bone and Soft Tissue Sarcoma)
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27 pages, 8184 KiB  
Review
Dedifferentiated Chondrosarcoma from Molecular Pathology to Current Treatment and Clinical Trials
by Weronika Zając, Julia Dróżdż, Weronika Kisielewska, Weronika Karwowska, Monika Dudzisz-Śledź, Agnieszka E. Zając, Aneta Borkowska, Anna Szumera-Ciećkiewicz, Bartłomiej Szostakowski, Piotr Rutkowski and Anna M. Czarnecka
Cancers 2023, 15(15), 3924; https://doi.org/10.3390/cancers15153924 - 01 Aug 2023
Cited by 1 | Viewed by 1725
Abstract
Dedifferentiated chondrosarcoma (DDCS) is a rare subtype of chondrosarcoma, a primary cartilaginous malignant neoplasm. It accounts for up to 1–2% of all chondrosarcomas and is generally associated with one of the poorest prognoses among all chondrosarcomas with the highest risk of metastasis. The [...] Read more.
Dedifferentiated chondrosarcoma (DDCS) is a rare subtype of chondrosarcoma, a primary cartilaginous malignant neoplasm. It accounts for up to 1–2% of all chondrosarcomas and is generally associated with one of the poorest prognoses among all chondrosarcomas with the highest risk of metastasis. The 5-year survival rates range from 7% to 24%. DDCS may develop at any age, but the average presentation age is over 50. The most common locations are the femur, pelvis humerus, scapula, rib, and tibia. The standard treatment for localised disease is surgical resection. Most patients are diagnosed in unresectable and advanced stages, and chemotherapy for localised and metastatic dedifferentiated DDCS follows protocols used for osteosarcoma. Full article
(This article belongs to the Special Issue Advances in Diagnosis and Treatment for Bone and Soft Tissue Sarcoma)
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