Myxofibrosarcoma (fibromyxosarcoma) is one of the most common soft tissue sarcomas in elderly patients, yet population-based data characterizing its epidemiology and outcomes remain limited. This study aimed to characterize the clinicopathological features, treatment patterns, and survival outcomes of fibromyxosarcoma using the Surveillance, Epidemiology, and End Results database. Patients diagnosed with myxofibrosarcoma between 1992 and 2022 were identified from the Surveillance, Epidemiology,...
Author: Akef Obeidat
Spinal intradural extramedullary Ewing's sarcoma is extremely rare in children. This case involves a 15-year-old boy with walking difficulties, successfully treated with total resection, confirmed by postoperative MRI. Following 20 months of being asymptomatic, he developed headaches due to obstructive hydrocephalus from a third ventricle lesion, which was biopsied and confirmed as Ewing's sarcoma. This case represents the third documented instance of brain metastasis from intradural...
Author: Manuja Pimplodkar
Osteosarcoma (OS) is the most common primary bone cancer in adolescents and young adults. Despite tremendous preclinical and clinical efforts to advance therapy for OS, the standard of care, consisting of surgical resection and pre- and postoperative chemotherapy, has remained unchanged for over 40 years. Growing molecular understanding of OS highlights tumor heterogeneity as a major obstacle to therapeutic advances. In this narrative review, we comprehensively discuss current evidence of OS...
Author: Sukjoo Cho
Radiotherapy is one of the most effective methods of cancer treatment. New, more effective, and safer radiotherapy methods can be developed thanks to selective radioprotectors. In our study, we investigated the antioxidant and radiomodulatory activity of sodium aminodihydrophthalazinedione (the drug Tameron^(®)) on human mesenchymal stem cells (MSCs) and human osteosarcoma cells of the MNNG/Hos line in vitro. We have shown that sodium aminodihydrophthalazinedione effectively scavenged...
Author: Artem Ermakov
Molecular factors influencing prognosis in leiomyosarcomas (LMS) remain poorly understood. Given that LMS arise from smooth muscle and that the dystrophin gene (DMD) has been suggested as a tumor suppressor in this tumor type, this study investigated dystrophin expression and somatic mutations in DMD, and dystrophin-glycoprotein complex (DGC) and dysferlin (DYSF) coding genes as prognostic biomarkers in LMS. Seventy-seven patients with LMS were retrospectively included in the study. Dystrophin...
Author: Juliana Salazar
Central chondrosarcoma is the second most common primary malignant bone tumour, and grade progression markedly worsens prognosis. The contributions of lipid metabolic reprogramming and epigenetic co-dysregulation to grade progression remain poorly characterised. We integrated a bulk RNA-seq discovery cohort of 53 graded central chondrosarcomas (GSE299759) with a single-cell analysis of eight chondrosarcomas (GSE184118). Because the atypical cartilaginous tumour (ACT) and dedifferentiated groups...
Author: Batuhan Ayhan
Therapeutic agents targeting the PD1-PDL1 interaction, commonly called PD1 blockade, are of great clinical value; however, predicting which patients will benefit has been inconsistent, in part, due to a lack of reliable biomarkers. Quantifying PD1 saturation by PDL1 in tumor tissue has the potential to serve as a biomarker; unfortunately, few diagnostic technologies are available to reliably quantify PD1 saturation in clinical biospecimens. Here, we report on a novel bioassay based on RNA...
Author: Suresh Veeramani
This study aimed to investigate the expression of immune checkpoint-associated proteins in phyllodes tumors (PTs) and assess their clinicopathologic and prognostic significance. Surgical resection specimens from 200 patients were included, and the expressions of CD24, Siglec-10, CD47, and SIRPα in both the epithelial and stromal components of the tumor were assessed, with ≥1% positivity considered positive. Of 200 cases, 145 were benign, 44 borderline, and 11 malignant. The expressions of CD24,...
Author: Eunah Shin
Iron overload disrupts cellular homeostasis and drives ferroptosis through dysregulated iron metabolism. Non-coding RNAs (ncRNAs) are considered as key regulators of various biological functions and targets for a new generation of RNA therapeutics and biomarkers. However, few studies have investigated the regulatory roles of ncRNAs, particularly competitive endogenous RNAs (ceRNAs) in iron overload. This study performed whole-transcriptome sequencing to characterize the ceRNA network in ferric...
Author: Yifan Teng
Background and Objectives: Uterine carcinosarcoma (UCS) is a rare and highly aggressive gynecologic malignancy with poor clinical outcomes and limited therapeutic options. This study investigated the prognostic significance of molecular subgroups defined by p53 expression and mismatch repair (MMR) status in UCS. Materials and Methods: This retrospective study included 51 patients with uterine carcinosarcoma who underwent surgical treatment between 2010 and 2023. Immunohistochemical analyses were...
Author: Emine Kilic Bagir
CONCLUSIONS: HHV-8 PCR in archived FFPE KS biopsies shows high specificity and good sensitivity and acts as a complementary diagnostic tool with fair agreement with histopathology. It is particularly valuable in diagnostic gray areas but should always be interpreted together with clinical, histopathological and immunohistochemical findings.
Author: Cemal Çiçek
Objectives: Chondrosarcoma is the most common type of primary bone sarcoma in adults with a high risk of local recurrence and metastasis. Chondrosarcomas are largely resistant to chemotherapy and radiotherapy, meaning that surgery is the mainstay of treatment for most patients. Therefore, new therapeutic targets are required. Cluster of differentiation 44 (CD44) is a transmembrane protein that has roles in cell proliferation, adhesion and migration and is shown to be overexpressed in several...
Author: Zoe Bell
Background: Osteosarcoma remains a biologically complex and clinically challenging malignancy, with survival gains plateauing despite decades of multimodal therapy incorporating surgery and cytotoxic chemotherapy. Unlike cancers in which mutation-centric precision oncology has yielded transformative advances, osteosarcoma is characterized by profound structural variation, copy number alteration dominance, and dynamic clonal evolution, limiting the effectiveness of single-target approaches. These...
Author: Srinivasan Vijayakumar
Breast sarcomas are mesenchymal tumors that constitute less than 1% of all breast malignancies and account for less than 5% of all soft-tissue malignant tumors. Various subtypes include angiosarcoma, liposarcoma, leiomyosarcoma, osteosarcoma, rhabdomyosarcoma, synovial sarcoma, and undifferentiated pleomorphic sarcoma. Staging and management of these malignancies differ from those for other more common breast malignancies such as carcinoma. The clinical outcome depends on various factors...
Author: Jorge L Huayanay Espinoza
Rhabdomyosarcoma (RMS) confined to the bone marrow represents an exceptionally rare and aggressive presentation that can mimic primary hematological malignancies, often leading to diagnostic delays and therapeutic challenges. We report the case of a 34-year-old woman who presented with clinical and laboratory findings highly suggestive of a hematological disorder, including cytopenias and diffuse bone marrow involvement. Initial evaluation raised suspicion for leukemia; however, comprehensive...
Author: Mohammad Hassan Hodroj
Background/Objectives: The most common soft tissue sarcomas in adults are liposarcomas, a heterogeneous group of malignant tumors. A structured literature search was conducted to better understand the clinical-imaging aspects and molecular behavior underlying the therapeutic approach. Methods: A scoping review was performed according to the PRISMA-ScR guidelines. Searches were conducted in MEDLINE, Web of Science Core Collection, and Google Scholar for the period of 1 January 2016-27 March 2026....
Author: Bogdan Marian Caraban
Myxoid epithelioid sarcoma is a rare morphologic variant of epithelioid sarcoma characterized by abundant myxoid stroma and frequent loss of SMARCB1/INI1 expression. Orbital involvement is exceptionally uncommon. We report a case of a SMARCB1-deficient malignant orbital neoplasm with features overlapping myxoid epithelioid sarcoma in a 61-year-old man presenting with visual disturbance. Imaging studies revealed a 2.5-cm medial orbital mass without evidence of another primary lesion. The tumor...
Author: Michiko Hosaka
BACKGROUND: Sarcomas are rare, diverse malignancies with limited therapeutic options and poor clinical outcomes. Preclinical models that preserve tumour biology are urgently needed to advance mechanistic understanding and functional precision oncology. We established and comprehensively characterized 29 early-passage patient-derived sarcoma cell (PDC) cultures from 19 patients, representing 11 sarcoma subtypes. Multi-region and multi-site sampling enabled generation of PDCs from spatially...
Author: Stefanie Gijsels
CONCLUSION: Fertility preservation may be possible in selected early-stage uterine adenosarcoma cases without high-risk features such as sarcomatous overgrowth or myometrial invasion, with thorough counseling and strict follow-up. Further research is needed to develop standardized protocols and improve management in this context.
Author: E Ziegler
We propose a mathematical model for the growth and treatment of Undifferentiated Pleomorphic Sarcoma (UPS) using a system of nonlinear differential equations. The model combines Gompertz-type tumor growth with surface-dependent necrotic loss, surgical resection with residual disease, postoperative recovery, tumor-immune interaction, and radiation treatment scheduling. We study the mathematical properties of the model and obtain several results. The growth equation shows the existence of a...
Author: Sumit Roy
CONCLUSIONS: In the reference case analysis, AC outperformed its use in terms of outcomes at 1 year, but a high level of willingness to pay would be required for AC to be cost-effective. At 3 and 5 years, AC was deemed to be not cost-effective for patients with non-metastatic sarcoma.
Author: Lionel Perrier
Osteosarcoma remains a major treatment challenge, especially for patients with recurrent, refractory or metastatic diseases. Adoptive cell therapy (ACT), including CAR-T cells, TCR engineered T cells, CAR-NK cells and macrophage based cell therapy, provides a promising strategy for redirecting immune effector cells to fight osteosarcoma. However, clinical translation has been limited by antigen heterogeneity, on-target/off-tumor toxicity, insufficient tumor trafficking, poor persistence,...
Author: Shiguo Zuo
Osteosarcoma, an aggressive malignancy of long bones in children and young adults, responds poorly to conventional chemotherapy, and distant metastasis remains the principal cause of death. Here we identify chemokine CXCL14 as a driver of epithelial-to-mesenchymal transition (EMT) and metastatic behavior. CXCL14 expression was markedly higher in osteosarcoma cells than in normal osteoblasts. Knockdown of CXCL14 significantly reduced cell migration and wound closure, accompanied by a shift from...
Author: Chun-Han Hou
CONCLUSION: Overall, our study demonstrated that KIF18A is a promising therapeutic target in osteosarcoma. The novel inhibitor AM-1882 offers exceptional anti-tumor efficacy paired with a favorable, low-toxicity safety profile.
Author: Qiang Yan
Objective: To investigate the surgical approach for the resection of complex tumors located on the lateral side of the pelvic floor. Methods: A retrospective analysis was conducted on 24 patients with complex tumors on the lateral side of the pelvic floor who were treated at The First Affiliated Hospital of Zhengzhou University from April 2023 to April 2025. Among the 24 patients, there were 16 cases of aggressive pelvic myxoma of the pelvic floor (including 10 recurrent cases), 6 cases of soft...
Author: L L Guo
CONCLUSION: Patients with MLS should undergo standard extended resection and individualized adjuvant radiotherapy and chemotherapy as soon as possible after unplanned surgery to significantly reduce the local recurrence rate. In view of the high risk of extrapulmonary metastasis, a comprehensive preoperative assessment of metastasis is required.
Author: Guoxin Qu
Giant cell tumor of bone (GCTB), osteosarcoma, and Ewing sarcoma represent the most characteristic primary bone neoplasms, and optimization of their diagnostic and therapeutic strategies remains a research priority in the field of bone and soft tissue oncology. The National Comprehensive Cancer Network (NCCN) released the Clinical Practice Guidelines in Bone Cancer (Version 2.2026), introducing key evidence-based updates spanning equivalent biological therapy alternatives, perioperative...
Author: Ruoyu Li
CONCLUSIONS: CM use is associated with differential efficacy outcomes in sarcoma patients receiving ICIs. These findings highlight the need for prospective studies to define the impact of commonly prescribed medications on ICI outcomes and to optimize clinical trial stratification.
Author: Adel Shahnam
BACKGROUND Retroperitoneal liposarcoma is a malignant soft tissue sarcoma arising from adipocyte precursors, with an incidence of 3-4 cases per million. Giant retroperitoneal tumors pose significant surgical challenges due to their size and proximity to vital structures. CASE REPORT We report the case of a 61-year-old woman who presented with progressive abdominal distension, mild discomfort, nausea, dyspepsia, and exertional dyspnea. Preoperative MRI revealed a giant retroperitoneal mass...
Author: Leon B Hajdari
CONCLUSIONS: Over one-third of AYAs with sarcoma met the primary endpoint at 1 year, with half of these abnormalities persisting at 2 years, primarily driven by LVWT/D ratio reductions. Subclinical changes in strain and diastolic function were observed, reflecting the broad cardiac impact of high-dose Dox in this population.
Author: Efstratios Koutroumpakis
CONCLUSION: Surgical resection of giant retroperitoneal tumors is feasible and can be performed safely with acceptable short-term outcomes when guided by careful preoperative planning and individualized operative strategy. Given the heterogeneity of tumor subtypes and limited follow-up, this case series should be interpreted as a demonstration of surgical feasibility and perioperative safety rather than oncologic efficacy. Tumor size and anatomical complexity remain the primary determinants of...
Author: Mahmoud Azhary
Haemangiosarcoma (HSA) is a highly aggressive vascular tumour in dogs, characterised by rapid growth, early metastasis, and poor prognosis despite conventional treatment with surgery and doxorubicin-based chemotherapy. A 14-year-old spayed female Maltese (4.14 kg) was presented with progressive abdominal distension and anorexia. Imaging revealed a large retroperitoneal mass, and computed tomography identified a 12.1 × 8.0 × 8.5 cm heterogeneous tumour. Exploratory laparotomy confirmed the lesion...
Author: Woo Dae Park
Alveolar soft part sarcoma (ASPS) is a rare malignant tumour that is considered to lack a normal cell counterpart and is commonly associated with a poorer prognosis. Etio-pathologically, it has been associated with alveolar soft part sarcoma critical region-1-transcription factor for immunoglobulin heavy chain enhancer 3 fusion gene. Diagnosis is usually confirmed through histopathology, immunohistochemistry (IHC) and molecular testing. Being chemo-resistant, surgical excision with clear margins...
Author: Misbah Mateen Kamble
The OX40/OX40L axis entered clinical development in atopic dermatitis with a strong biological rationale and early signs of durable activity. However, as the treatment landscape evolved, questions emerged about whether the magnitude of monotherapy benefit was sufficient relative to established and emerging therapies. The discontinuation of rocatinlimab after confirmed and suspected cutaneous Kaposi's sarcoma cases, together with two cumulative cases reported in the amlitelimab program in...
Author: Shahram Salek-Ardakani
CONCLUSIONS: Survivors of pediatric and young adult osteosarcoma exhibit a high success rate in achieving normal conception and childbirth, aligning with the general population. To inform pediatric and adolescent patients with osteosarcoma, as well as their parents, about the high success rate associated with achieving a normal conception and childbirth should be the standard.
Author: José María Lamo-Espinosa
CONCLUSION: Uterine carcinosarcoma exhibits minimal local invasion but high systemic aggressiveness. Traditional markers of local spread, such as myometrial invasion, are less predictive for CS. Systematic surgical staging remains essential, and multimodal adjuvant CRT is crucial to mitigating the inherent survival disadvantage of CS.
Author: Burcu Soyak
ObjectiveThis study aimed to establish a diagnostic nomogram for identifying synchronous lung metastasis at initial diagnosis in osteosarcoma patients, and to descriptively analyze overall survival patterns in patient subgroups.MethodsA total of 1149 eligible osteosarcoma cases diagnosed between 2010 and 2015 were retrieved from the Surveillance, Epidemiology, and End Results (SEER) database. Candidate predictors were screened by univariate logistic regression (p < 0.10) and entered into...
Author: Sisi Ge
CONCLUSION: iTRAC demonstrates superior prognostic utility in STS over the current grading systems, effectively stratifying metastatic risk for patients who might benefit from alternative therapeutic strategies. iTRAC holds the potential for personalizing chemotherapeutic approaches, paving the way for a new precision oncology approach in STS.
Author: Ataaillah Benhaddou
CONCLUSIONS: Understanding the key characteristics of primary clival bone lesions is essential for clinicians and neurosurgeons to select optimal diagnostic and therapeutic approaches, ultimately aiming to improve patient outcomes.
Author: Vinicius Trindade Gomes da Silva
Osteosarcoma (OS) is a highly aggressive primary bone malignancy that predominantly affects children and adolescents. Although surgical intervention and neoadjuvant therapy demonstrate efficacy in localized OS, postoperative survival rates remain suboptimal for patients with metastatic and recurrent OS. In recent years, immunotherapy has garnered considerable attention due to its promising efficacy across various solid tumors. However, the distinct immunosuppressive tumor microenvironment in OS...
Author: Xihan Wan
Ewing sarcoma is the second most common bone cancer in children and young adults. In 85% of patients, a translocation between chromosomes 11 and 22 results in a potent fusion oncoprotein, EWSR1::FLI1. EWSR1::FLI1 is the only genetic alteration in an otherwise unaltered genome of Ewing sarcoma tumors. The EWSR1 portion of the protein is an intrinsically disordered domain involved in transcriptional regulation by EWSR1::FLI1. The FLI portion of the fusion contains a DNA binding domain shown to...
Author: Ariunaa Bayanjargal
CONCLUSIONS: To our knowledge, this is among the first studies to investigate kidney dysfunction and its consequences in adult LS patients. In our cohort of surgically treated adult patients with LS of the extremities and trunk, kidney dysfunction was a frequent and clinically impactful complication. It was significantly associated with decreased overall survival, loss of muscle and adipose tissue in sequential CT morphometry assessments and progressive functional decline. Off note,...
Author: Julian Kylies
We describe an extremely rare clinical case of primary ovarian osteosarcoma (OS) in a 70-year-old woman, along with the diagnostic criteria for this condition. The patient underwent chemotherapy and has been under observation for 27 months. Currently, tumor progression with metastatic lesions of the peritoneum is observed. This case highlights the importance of further studies of primary ovarian OS. Ovarian OS is not included in the 2022 WhO classification of genital tumors, but such cases...
Author: O Manzhura
High-grade endometrial stromal sarcoma (HGESS) is a rare tumor that typically occurs over a wide age range, from 14 to 71 yr, often presenting with nonspecific clinical symptoms. This tumor group displays heterogeneous tumor morphology and a growing number of molecular alterations. The most common alterations are YWHAE::NUTM2 fusion and BCOR gene alterations, including fusions and internal tandem duplications. Here, we report a HGESS with a novel ING3::BCOR fusion. A 74-yr-old female presented...
Author: Istem Kose
Primary renal osteosarcoma (PRO) is a rare extraskeletal malignancy, accounting for less than 1% of soft-tissue sarcomas and fewer than 50 reported cases to date. We present the case of a 47-year-old male who presented with six months of progressive right flank pain and an abdominal lump. Initial imaging with plain radiography and contrast-enhanced computed tomography urography revealed a large, heterogeneously enhancing right renal mass characterised by extensive, confluent, dense intratumoral...
Author: Michael Kumar Sah
Berberine, curcumin, biochanin A, cucurbitacin E, and caffeic acid phenethyl ester (CAPE) are plant-derived compounds with long histories of use in traditional medicine for inflammatory and proliferative conditions. Their known capacity to modulate NF-κB signaling makes them candidates for anticancer investigation, particularly in mesenchymal malignancies such as fibrosarcoma, which arise in muscle-rich environments shared with normal myogenic tissue. To evaluate the selective anticancer...
Author: Justyna Radzka
Curcumin, a natural polyphenolic compound derived from turmeric, exhibits broad-spectrum anticancer activities, but its ability to induce pyroptosis in osteosarcoma remains unknown. Osteosarcoma is the most common primary malignant bone tumor in children and adolescents, and novel therapeutic strategies are urgently needed to overcome osteosarcoma chemoresistance. Aim: This study aimed to investigate whether curcumin induces pyroptosis-associated molecular changes in human osteosarcoma cells and...
Author: Keqing Yuan
The field of immunotherapy for unresectable soft tissue sarcomas (STS) is transitioning from a one-size-fits-all approach toward a precision immuno-oncology paradigm. The pronounced immunological heterogeneity among histological subtypes and the limited predictive capacity of traditional classification underscore the imperative for immune-based stratification. Approximately 20% of STS exhibit an immune-activated tumor microenvironment, characterized by robust cytotoxic T lymphocyte infiltration,...
Author: Simo Zou
Soft tissue sarcomas are aggressive malignancies with limited responses to immunotherapy. Strategies that induce viral mimicry and type I interferon (IFN-I) signaling may enhance tumor immunogenicity but remain poorly explored in sarcoma. Here, we show that the CDK4/6 inhibitor abemaciclib triggers viral mimicry and robust IFN-I responses in sarcoma cells, leading to tumor growth suppression and remodeling of the tumor microenvironment toward an immune-permissive state. However, sarcoma cells...
Author: Jin-Fen Xiao
INTRODUCTION: Ewing sarcoma (ES) is the second most common malignant bone and soft tissue tumor in children, adolescents, and young adults. Current consensus guidelines, consistent with Children's Oncology Group practice, support the universal use of full-body fluorodeoxyglucose positron emission tomography (FDG-PET) and recommend reserving bone marrow aspiration and biopsy for cases in which FDG-PET raises concern for marrow involvement, leaving final decisions to the treating physician....
Author: Rachel Offenbacher