Bone and Soft Tissue Sarcomas: Current Challenges and Future Directions

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Oncology".

Deadline for manuscript submissions: closed (28 February 2023) | Viewed by 4747

Special Issue Editors


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Guest Editor
Sarcoma Department, Moffitt Cancer Center, Tampa, FL, USA
Interests: soft tissue sarcomas; bone sarcomas; surgical outcomes
Special Issues, Collections and Topics in MDPI journals

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Co-Guest Editor
Sarcoma Department, Moffitt Cancer Center, Tampa, FL, USA
Interests: bone and soft tissue sarcomas; limb preserving surgeries

Special Issue Information

Dear Colleagues,

Bone and soft tissue sarcomas are rare cancers that may imply high morbidity and poor disease specific survival despite current treatment modalities. While these tumors typically share a mesenchymal stem cell origin, they are typically characterized by tremendous genetic heterogeneity. For sarcomas as a whole, surgery is generally the mainstay of treatment, though adjuvant therapies typically differ depending on histologic type. For patients with osteosarcoma, chemotherapy dramatically improves survival rates; however, advances in adjuvant treatments have stagnated over the past 30–40 years. For chondrosarcoma, appropriate diagnosis and management continues to be a challenge and a continued subject of debate. In contrast, soft tissue sarcoma is a general term that encompasses over 70 histological subtypes. Radiation therapy is typically a component of the management of these tumors, though novel technologies such as proton beam therapy and carbon ion therapy hold additional promise. Owing to their collective rarity, research on sarcomas is inherently challenging; however, continued collaborative scientific efforts have helped to push the field forward.

This Special Issue aims to update the current challenges and future directions in the diagnosis, treatment and overall management of bone and soft tissue sarcomas in order to highlight the multidisciplinary approaches, personalized treatment strategies, and promising therapeutic approaches to the treatment of these challenging disease entitites. Continued research is needed to identify current obstacles and explore novel solutions to help optimize patient care, with a focus on how the integration of different treatment modalities can improve patient outcomes. Some of these areas include the development of improved models of sarcoma in the laboratory space, such as 3D organoids and comparative oncology, the investigation of liquid biopsy techniques and cDNA, the development of novel drug delivery mechanisms, the identication of novel drug targets, investigations into the immune system interaction in sarcoma, the potential role of immunotherapy, and explorations of how surgical advances, such as customized 3D printed implants, intraoperative margin detection, and navigated tumor resections, can improve outcomes in patients with these tumors.

Dr. Alexander L. Lazarides
Dr. Odion T. Binitie
Guest Editors

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Keywords

  • sarcoma
  • bone sarcoma
  • soft tissue sarcoma
  • osteosarcoma
  • liquid biopsy
  • immunotherapy
  • surgical outcomes
  • radiation therapy
  • chemotherapy

Published Papers (3 papers)

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Research

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11 pages, 2516 KiB  
Article
Tibia Multiplanar Deformities and Growth Disturbance Following Expandable Endoprosthetic Distal Femur Replacement
by Ahmad Shehadeh, Muhamad Al-Qawasmi, Omar Al Btoush and Zeinab Obeid
J. Clin. Med. 2022, 11(22), 6734; https://doi.org/10.3390/jcm11226734 - 14 Nov 2022
Cited by 2 | Viewed by 1541
Abstract
Background: Expandable distal femur endoprosthesis (EDFE) is commonly used to compensate for the loss of the distal femoral epiphyseal plate in skeletally immature children who have undergone surgical resection of bone malignancies. However, the effect of the passive tibial component of the EDFE [...] Read more.
Background: Expandable distal femur endoprosthesis (EDFE) is commonly used to compensate for the loss of the distal femoral epiphyseal plate in skeletally immature children who have undergone surgical resection of bone malignancies. However, the effect of the passive tibial component of the EDFE on tibial growth has not been extensively studied in the literature. This study aims to delineate the type, frequency, and associated risk factors of multiplanar proximal tibial deformities in skeletally immature children following the use of the expandable distal femur endoprosthesis (EDFE). Moreover, we plan to detect how these deformities influence the long-term functionality of the endoprosthesis in defining the need for subsequent implant revision or further surgical management. Patients and Methods: A total of 20 patients aged (7–12) years underwent expandable distal femur replacement. Two types of implants were used: Juvenile Tumor System (JTS) non-invasive prosthesis in 14 patients, and Modular Universal Tumor and Revision System (MUTARS)® Xpand Growing Prostheses in six patients. A scanogram and CT scan documented the measurements of longitudinal and multiplanar growth as leg length discrepancy (LLD), femur length discrepancy (FLD), tibia length discrepancy (TLD), and the yield values of rotational, sagittal, and coronal deformities of the tibia. The patients were followed up to assess the need for further management. Sex, age, size of tibial plate perforation, and type of implant used were studied for possible correlation with deformities or growth disturbance. Results: The patients were followed up for a mean of 3 (2–7) years. A total of 14 patients, (10 JTS, 4 implant cast) had a tibial deformity and/or growth disturbance. A single patient was found to have all deformities (growth, rotational, coronal, and sagittal). Fourteen patients were found to have an LLD ranging from 5.3 to 59 mm (median 21 mm), 12 had a TLD from 3 to 30 mm, (median 10 mm), and 11 patients showed evidence of malrotation from 6 to 32 degrees (median 11 degrees). TLD was found to contribute entirely to LLD in three patients, and >50% of LLDs in seven patients. All LLDs were treated conservatively, except in three patients; two received contralateral tibia epiphysiodesis and one received revision with a new implant. A single patient had a posterior tibia slope angle (PTSA) of −2.8 degrees, and three patients had a coronal deformity with a mean medial proximal tibia angle (MPTA) of 80.3 (77–83 degrees). Conclusions: Tibial growth disturbance and multiplanar deformities occur in the majority of patients following EDFE replacement, exacerbating LLD. Yet, these disturbances may be well tolerated, managed conservatively, and rarely mandate endoprosthetic revision or subsequent corrective surgery. Age at the time of surgery was found to be the only significant contributor to the development of tibia growth disturbance. Full article
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Review

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10 pages, 572 KiB  
Review
Isolated Limb Infusion for Limb-Threatening, Unresectable Sarcoma: Past Progress, Current Applications, and Future Directions
by Danielle K. DePalo and Jonathan S. Zager
J. Clin. Med. 2023, 12(12), 4036; https://doi.org/10.3390/jcm12124036 - 13 Jun 2023
Viewed by 941
Abstract
Treatment of soft tissue sarcomas (STSs) is complicated by disease heterogeneity. Further, it has not benefitted much from the recent therapeutic advances in other soft tissue malignancies. Surgical resection remains the gold standard in resectable disease, but unresectable, locally advanced STS requires alternative [...] Read more.
Treatment of soft tissue sarcomas (STSs) is complicated by disease heterogeneity. Further, it has not benefitted much from the recent therapeutic advances in other soft tissue malignancies. Surgical resection remains the gold standard in resectable disease, but unresectable, locally advanced STS requires alternative and multimodal approaches. Isolated limb infusion (ILI) provides regional chemotherapy to extremity STS and offers the potential for limb salvage. Despite being in use for nearly 3 decades, there is limited literature on ILI in STS. This review provides an overview of patient eligibility, the procedure, significant publications in this field, and opportunities for further progress. Full article
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11 pages, 840 KiB  
Review
Endoprosthetic Reconstruction in Ewing’s Sarcoma Patients: A Systematic Review of Postoperative Complications and Functional Outcomes
by Jude Abu El Afieh, Marena Gray, Matthew Seah and Wasim Khan
J. Clin. Med. 2022, 11(15), 4612; https://doi.org/10.3390/jcm11154612 - 8 Aug 2022
Cited by 4 | Viewed by 1729
Abstract
Ewing’s sarcoma (ES) is a rare primary bone cancer managed by radiotherapy, chemotherapy and surgical resection. The existing literature on limb salvage surgery with endoprostheses combines data for ES patients with osteosarcoma. This review aimed to evaluate surgical and functional outcomes of endoprosthetic [...] Read more.
Ewing’s sarcoma (ES) is a rare primary bone cancer managed by radiotherapy, chemotherapy and surgical resection. The existing literature on limb salvage surgery with endoprostheses combines data for ES patients with osteosarcoma. This review aimed to evaluate surgical and functional outcomes of endoprosthetic reconstruction in exclusively Ewing’s patients. We believe that this is the first comprehensive review to evaluate the outcomes of limb salvage surgery with endoprostheses exclusively in Ewing’s sarcoma patients. Clinical data and outcomes were collected from PubMed, Embase, Medline and Scopus. The inclusion criteria were studies on limb salvage surgery in ES patients, where individual patient data was available. Seventeen studies with a total of 57 Ewing’s patients were included in this review. Fifty-three of the ES patients preserved the limb after limb salvage with endoprostheses. The average five-year implant survivorship was 85.9% based on four studies in this review. Postoperative complications were categorised by Henderson’s failure modes. Soft tissue failure was the most common, occurring in 35.1% of patients, followed by deep infection in 15.7% of patients. There was a suggestion of ‘good’ functional outcomes with limb salvage surgery. The salient limitation of this review is the variability and rarity of the patient population. Homogenous data in a larger population is necessary to provide more insight into outcomes of limb reconstruction in ES. Full article
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