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Multimodality Management of Sarcomas (2nd Edition)

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

Deadline for manuscript submissions: 31 May 2026 | Viewed by 2120

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Department of Surgical Oncology, Medical School of Crete University Hospital, Heraklion, Greece
Interests: sarcoma; melanoma; breast cancer; colorectal cancer; peritoneal carcinomatosis; intraperitoneal chemotherapy; HIPEC
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Special Issue Information

Dear Colleagues,

This Special Issue is the second edition of "Multimodality Management of Sarcomas".

Due to their rarity and heterogeneity, with a wide variety of histological subtypes, biological behaviors, and tumor localization, the optimal management of sarcomas remains challenging. The treatment of sarcomas has to be individualized and typically consists of a combination of modalities. Currently, sarcomas are managed in a multimodal manner.

Initial appropriate imaging is of the utmost importance to demonstrate the exact localization and extension of the tumor, as well as its relation to adjacent structures, in order to facilitate the performance of a diagnostic core needle biopsy and the planning of a surgical procedure when systemic disease is excluded. A preoperative biopsy is of the utmost importance to differentiate between benign and malignant tumors and to define the sarcoma subtype. The pathological examination of those rare and heterogeneous sarcoma tumors is very demanding and a significant level of expertise is required. The treatment plan for each sarcoma is personalized according to its imaging and pathology.

Until recently, most extremity soft tissue sarcomas were locally treated with amputation; currently, the majority of patients receive a limb-salvage treatment with a wide excision of the tumor, along with radiotherapy. Current evidence shows that preoperative radiotherapy may be preferred over the typical postoperative radiotherapy, whereas in low-risk patients, radiotherapy may even be omitted. In selected cases, preoperative systemic chemotherapy or isolated limb perfusion may be indicated in an attempt to preserve the extremity. For retroperitoneal sarcomas, recently, a more aggressive surgical approach has been advocated for, whereas the exact role of preoperative radiotherapy or chemotherapy to facilitate resection and to reduce the high risk of local recurrence has still to be defined. The systemic therapy of soft tissue sarcomas, either as an adjuvant treatment or for metastatic disease, has evolved from the classical anthracycline-based regimen to a more individualized histology-driven chemotherapy, whereas novel drugs have been developed. Bone sarcomas are also usually managed in a multimodal manner, depending on the histological type. Although localized chondrosarcomas are initially treated by surgical excision and/or radiotherapy, Ewing sarcomas are traditionally treated with systemic chemotherapy, followed by local treatment, including surgery and/or radiotherapy, as well as the continuation of systemic chemotherapy. Similarly, osteosarcomas are usually treated with a combination of chemotherapy and surgery, with or without radiotherapy, and the continuation of chemotherapy.

As has been illustrated, the management of sarcomas is challenging and multimodal, including radiology and pathology for initial diagnosis and, subsequently, surgery, as well as the use of chemotherapy and/or radiotherapy for definite treatment, in order to achieve optimal oncological and functional outcomes. The various aspects of this multimodal management technique and its current role will be discussed in this Special Issue.

Prof. Dr. Eelco de Bree
Guest Editor

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Keywords

  • sarcomas
  • multimodal
  • pathology
  • radiology
  • surgery
  • chemotherapy
  • radiotherapy

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Related Special Issue

Published Papers (2 papers)

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Research

24 pages, 9188 KB  
Article
Clinical Presentation and Tumour Burden in Head and Neck Sarcomas: Implications for Early Diagnosis and Referral
by Samhita Shanmugasundaram, Malla Salli, Amrita Jay, Antonia Timmis, Xin Kowa, Simon Morley, Katrina Ingley, Rachael Windsor, Ajla Wasti, Harini Rao, Franel Le Grange, Sandra J. Strauss, Vasilios Karavasilis, George Bitar, Simon Wan, Jonathan Joseph, Nicholas Kalavrezos and Deepti Sinha
Cancers 2026, 18(8), 1298; https://doi.org/10.3390/cancers18081298 - 20 Apr 2026
Viewed by 592
Abstract
Background/Objectives: Head and neck sarcomas account for 11% of all soft tissue and 9% of all bone sarcomas in the UK. Diagnostic delays are common, with non-specific symptoms and histological misdiagnosis reported in up to 42% of cases. This study aims to evaluate [...] Read more.
Background/Objectives: Head and neck sarcomas account for 11% of all soft tissue and 9% of all bone sarcomas in the UK. Diagnostic delays are common, with non-specific symptoms and histological misdiagnosis reported in up to 42% of cases. This study aims to evaluate the association between presenting symptoms, symptom duration, and tumour size to inform a tailored HNS diagnostic strategy for early referral to a tertiary centre. Methods: We analysed a retrospective cohort of 425 adult and paediatric patients referred to the London Sarcoma Service between 2002 and 2025. Results: Our cohort analysis identified a median tumour size of 44.00 mm and symptom duration of 3 months. Although symptom duration did not predict tumour size (β = 0.63, p = 0.76), non-specific symptoms (swelling, pain, nasal/oral changes) were significantly associated with larger tumours (OR 1.96–3.66), alongside systemic symptoms (β = 22.90 mm, p = 0.044). Each 1 mm increase in tumour size was also associated with a 2.60% increased chance of a higher-grade sarcoma (OR = 1.03 per mm, p < 0.001). Conclusions: To our knowledge, this is the largest cohort study to characterise diagnostic patterns in HNS. Our findings reveal three critical insights: 1. Current size-based referral thresholds are inadequate. 2. Non-specific symptoms, such as nasal or oral symptoms, are frequently overlooked. 3. The anatomical complexity of the HN region demands early tailored diagnostic strategies. We propose a hypothesis-generating ‘1–2–1’ framework to support earlier clinical suspicion, which requires prospective validation. Full article
(This article belongs to the Special Issue Multimodality Management of Sarcomas (2nd Edition))
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12 pages, 1100 KB  
Article
Primary Cardiac Sarcomas: Clinical Characteristics, Management, and Outcomes at a Spanish National Reference Center
by Carlos López-Jiménez, Mónica Benavente de Lucas, Ana Gutiérrez-Ortiz de la Tabla, Natalia Gutiérrez Alonso, Marta Arregui and Rosa Álvarez
Cancers 2025, 17(24), 3947; https://doi.org/10.3390/cancers17243947 - 10 Dec 2025
Viewed by 1142
Abstract
Background/Objectives: Primary cardiac sarcomas are rare and aggressive tumors. Management is often guided by evidence from other sarcoma types due to limited disease-specific data. This study aimed to analyze the clinical characteristics, pathology, treatment, and outcomes of primary cardiac sarcomas at a national [...] Read more.
Background/Objectives: Primary cardiac sarcomas are rare and aggressive tumors. Management is often guided by evidence from other sarcoma types due to limited disease-specific data. This study aimed to analyze the clinical characteristics, pathology, treatment, and outcomes of primary cardiac sarcomas at a national referral center in Spain. Methods: We conducted a retrospective, observational, single-center study from February 2017 to December 2024. Patient data were collected from medical records, and a descriptive analysis was performed. Results: Twelve patients were identified (58% female; median age 43 years, range 13–76). Dyspnea was the most common symptom (8/12, 67%), and the right atrium was the most frequent tumor site (6/12, 50%). Angiosarcoma was the predominant histologic subtype (6/12, 50%). Seven patients had localized disease at diagnosis. Surgery was performed in six patients, with complete (R0) resection in two. Two patients received adjuvant chemotherapy, one underwent cardiac transplantation, and one received sequential chemo- and radiotherapy. All patients experienced tumor recurrence, with a median recurrence-free survival of 5 months (95% CI, 1.5–8.6). Median overall survival for localized disease was 22 months (95% CI, 16–28). Five patients were metastatic at diagnosis, and 11 of 12 developed metastases. Median progression-free survival for first-line therapy was 5.9 months (95% CI, 1.8–9.9), and median overall survival for advanced disease was 12 months (95% CI, 10–13.6). Conclusions: Complete surgical resection was rarely achieved, and recurrence was universal. Outcomes remained poor even for localized disease, highlighting the limited efficacy of current therapies and the need for improved multimodal treatment strategies. Full article
(This article belongs to the Special Issue Multimodality Management of Sarcomas (2nd Edition))
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