Soft Tissue/Sarcoma -Pubmed

Osteosarcoma (OS) is a highly aggressive mesenchymal bone tumor with a dismal prognosis, and its underlying molecular mechanisms remain elusive. Ribosomal protein L13a (RPL13a) contributes to tumorigenesis, while methyltransferase-like 14 (METTL14), a core m⁶A methyltransferase, modulates OS progression via epigenetic regulation. Our prior work identified the PTEN/PI3K/AKT pathway as a key OS regulator. This study aimed to clarify the regulatory interplay among METTL14, RPL13a and PTEN/PI3K/AKT...

Expression of vascular endothelial growth factor receptor 2 (VEGFR2/KDR) is elevated in Kaposi Sarcoma lesions and is a key driver of vasculogenesis and angiogenesis. Increased levels of KDR are associated with several cancers and has been linked to the oncogenic potential of Kaposi sarcoma herpesvirus (KSHV)-infected cells. However, the functional role of KDR in KSHV infection requires further exploration. Here, we have demonstrated the importance of KDR expression in KSHV infection and...

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Background: Pediatric sarcomas are aggressive malignancies characterized by marked biological heterogeneity and a high risk of relapse. Standard surveillance relies on imaging and invasive biopsies, which may fail to detect early molecular disease. Liquid biopsy using circulating tumor DNA (ctDNA) and microRNAs offers a minimally invasive strategy for longitudinal monitoring. This study aimed to evaluate dynamic changes in ctDNA and circulating microRNAs during treatment and examined their...

Rhabdomyosarcoma with alveolar morphology is a diagnostically challenging tumor pattern, particularly in cases lacking canonical FOXO1-associated fusions. In the current WHO framework, molecular fusion status plays a central role in classification and risk stratification. However, a subset of tumors lacks canonical rearrangements and harbors alternative genetic events. We report a case of a 4-year-old girl with a massive parameningeal rhabdomyosarcoma presenting with cranial nerve dysfunction...

Pulmonary spindle cell tumors are aggressive neoplasms with limited systemic treatment options, although a subset may harbor actionable genomic alterations. Because conventional fusion assays may miss rearrangements involving atypical or previously uncharacterized partners, we systematically investigated oncogenic fusions in pulmonary spindle cell tumors using anchored multiplex PCR-based targeted RNA sequencing. Formalin-fixed, paraffin-embedded tumor samples from 11 surgically resected...

CONCLUSION: This case highlights the differing clinical manifestations of KSHV and the efficacy of immunotherapy in KSHV-associated primary effusion lymphoma.

Conventional size-based criteria have limitations in accurately assessing neoadjuvant therapy (NAT) response in soft tissue sarcomas (STS). This systematic review evaluated the association between dynamic contrast-enhanced MRI (DCE-MRI) parameters and histopathology-based response to NAT in STS and summarized which DCE-MRI parameters show the most consistent associations across diverse clinical contexts. Following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA)...

A 10.5-year-old male castrated Standard Poodle was referred for the evaluation of chronic C6-T2 myelopathy. MRI revealed a severe asymmetric enlargement of the ventral internal vertebral venous plexus, intervertebral/vertebral veins, and possibly the C6 basivertebral vein, with secondary mild spinal cord compression. Intravascular neoplasm, particularly lymphoma, with probable concurrent thrombus formation, was prioritized. Due to the poor prognosis and subsequent development of severe anemia...

CONCLUSION: This study reveals that during the progression of OS from a nonmetastatic to a metastatic state, BAMBI expression is downregulated and may be regulated by JUN. The decreased expression of BAMBI leads to hyperactivation of the TGF-β signaling pathway, thereby promoting the migration and invasion of OS cells. These findings provide potential immune-related and molecular targets that could inform single cell-guided strategies for targeted therapy and overcoming resistance.

Immunotherapy based on the adoptive cell transfer of tumor-infiltrating lymphocytes (TILs) has proven effective in treating human metastatic melanoma patients, but success in tumors with lower mutational burdens remains a challenge. Preclinical evaluation of cellular therapies commonly relies on murine models, which often require implantation of tumors into immunocompromised mice and thus do not accurately reflect the complex tumor-immune interactions seen in patients. Alternatively, spontaneous...

CONCLUSIONS: Intraoral involvement by ES may infrequently occur either in the setting of primary or metastatic disease. The epithelioid, spindled, and, occasionally, rhabdoid cytomorphology of ES in conjunction with the aberrant immunoexpression of cytokeratins and EMA may cause a major diagnostic pitfall in oral biopsy specimens. A broad panel of epithelial and non-epithelial immunohistochemical markers, including SMARCB1, together with evaluation for surface dysplasia and a detailed clinical...

CONCLUSION: This study demonstrates that autophagy affects the anticancer activity of PARPi in Ewing sarcoma cells.

CONCLUSIONS: In R0-resected, non-metastatic chondrosarcoma, both preoperative sarcopenia and postoperative declines in muscle and adipose tissue, particularly SMI loss ≥ 30% and VAT loss ≥ 25%, are independent predictors of reduced survival and worse postoperative outcomes. Leveraging routinely acquired staging CTs for morphometric analysis may enable early risk identification and guide personalized perioperative management.

CONCLUSION: This study provides the first systematic analysis of dynamic changes in peripheral blood lymphocyte subsets in metastatic OSA patients undergoing ICIs. Our findings suggest that elevated proportions and dynamic increases of peripheral blood CD3^(-)CD56^(+) (NK cells) and CD8^(+)HLA-DR^(+) (activated cytotoxic T cells) after ICIs are consistently associated with improved OS and better treatment response. These accessible biomarkers hold potential for predicting ICIs efficacy and...

We piloted teledermatology to support detection of skin conditions, including Kaposi sarcoma (KS), at HIV clinics in Kenya and Uganda. Dermatologists remotely reviewed clinical information and images submitted by providers via a mobile platform. Of 273 consults, 18 were suspected of KS; 9 were biopsied, 7 were diagnosed as KS, and 4 received KS-specific treatment. Teledermatology supported identification of KS; however, biopsy follow-up was limited, highlighting the need for integrated service...

A woman in her 60s with a prior history of cervical carcinoma treated with chemoradiation presented with a polypoidal vaginal lesion nearly 7 years after initial therapy. Histopathology confirmed osteosarcoma confined to the posterior vaginal wall, with imaging excluding metastases. Total vaginectomy was performed, and final diagnosis was radiation-induced vaginal osteosarcoma. Although adjuvant chemotherapy was recommended, the patient declined. 6 months later, local recurrence and lung...

We report the case of a woman with metastatic alveolar soft part sarcoma (ASPS) who experienced pregnancy-associated disease progression followed by unexpected postpartum regression. Pulmonary lesions increased in size after a miscarriage and during a subsequent pregnancy, prompting consideration for systemic treatment and potential enrolment in a clinical trial evaluating a tyrosine kinase inhibitor in combination with an immune checkpoint inhibitor. However, baseline staging CT performed a few...

Canine osteosarcoma (OSA) is a highly aggressive primary bone tumor and a valuable model in comparative oncology. Nevertheless, commonly used canine in vitro models remain incompletely and inconsistently characterized, while exhibiting substantial biological heterogeneity affecting experimental outcomes. This study aimed to comparatively characterize three canine osteosarcoma cell lines (OSCA8, OSCA29, and D17) in reference to canine hTERT fibroblasts, and with a focus on functional properties...

CONCLUSIONS: Our analysis suggests that broad genomic profiling may provide complementary molecular information in diagnostically challenging cases managed at specialised sarcoma centres, particularly when morphology and immunohistochemistry are insufficient. In the present series, however, the detected rearrangements did not alter systemic treatment, and the data do not support claims of prognostic, predictive, or therapeutic actionability.

Phyllodes tumours (PTs) of the breast are rare fibroepithelial neoplasms with potentially aggressive behaviour, characterised by rapid growth, a significant risk of local recurrence, and occasional metastatic spread. Optimal management remains controversial, particularly regarding surgical margins, adjuvant radiotherapy, and the relevance of molecular markers in predicting tumour behaviour. A PRISMA 2020-guided qualitative systematic review was conducted of studies published between January 2000...

Background/Objectives: Kaposi's sarcoma (KS) is an angioproliferative malignancy caused by Kaposi's sarcoma-associated herpesvirus (KSHV), characterized by aberrant angiogenesis, chronic inflammation, and endothelial cell transformation. Given the multi-factorial nature of KS pathogenesis, strategies that simultaneously modulate multiple mo-lecular targets are considered more promising than single-target approaches. However, effective multi-target therapeutic agents for KS remain limited,...

CONCLUSION: Ybx1 is a potential target molecule of the novel anti-metastatic coumarin-based compound SSKP-0076.

CONCLUSION: 5-ALA RDT demonstrated significant anti-tumor effects in both in vitro and in vivo osteosarcoma models. These findings suggest its potential as a therapeutic approach for osteosarcoma.

CONCLUSION: In this retrospective exploratory analysis, tumor size and the use of carbon-ion radiotherapy were associated with survival in patients with pelvic sarcomas. Large tumor size was linked to unfavorable outcomes, whereas CIRT was associated with improved survival. Although limited by the small sample size and potential selection bias, these findings suggest that CIRT may represent a potential treatment option for selected patients with pelvic sarcomas and highlight the importance of...

BRAF fusions are rare but recurrent driver events in a spectrum of mostly pediatric and young adult mesenchymal soft tissue tumors, including infantile fibrosarcoma, lipofibromatosis-like neural tumors, and other kinase fusion spindle cell neoplasms. Their clinicopathologic spectrum, co-occurring secondary genomic events, and frequency among various sarcoma histotypes remain incompletely characterized. The purpose of this study is to comprehensively investigate the incidence and structural...

Recurrent fusions involving FGFR1-4 genes have been previously described in rare subsets of mostly benign chondroid and mesenchymal neoplasms involving bone and soft tissue. However, a more comprehensive analysis of sarcomas associated with FGFR fusions, including their incidence and histotypes, has not been performed. Triggered by an FGFR1-rearranged unclassified high-grade sarcoma with myogenic differentiation, we investigated our molecular database for sarcomas with FGFR gene fusions to...

This review addresses the treatment of malignant musculoskeletal tumours associated with significant late effects. Surgical resection and reconstruction may result in chronic pain, reduced muscle strength, limited joint mobility, mechanical failure of implants, and need for reoperation. Radiotherapy increases the risk of wound complications, fibrosis, fractures, and secondary malignancies, while chemotherapy may cause cardiac, renal, and skeletal toxicity. Amputation and limb-sparing procedures...

Maintenance chemotherapy prolongs progression-free survival (PFS) in several malignancies, but its role in soft tissue sarcoma (STS) remains unclear. Conventional doxorubicin is limited by cumulative toxicities. Liposomal doxorubicin may allow prolonged therapy. We evaluated outcomes of patients treated with maintenance liposomal doxorubicin following induction doxorubicin. This was a single-center retrospective study using the Quebec Sarcoma Registry from 2015 to 2025. Eligible patients had...

Extremity soft tissue sarcoma resection often results in complex defects requiring reconstruction to preserve function and support multidisciplinary treatment. Propeller flaps have emerged as a local alternative to free flaps in selected cases, but their role in sarcoma reconstruction remains incompletely defined. This systematic review aimed to assess the current evidence on the indications, reconstructive outcomes, and oncologic reporting of propeller flaps in extremity sarcoma reconstruction....

» Pelvic Ewing sarcoma presents unique challenges as pelvic tumors can grow to large sizes before detection and can require complex reconstructionsaffecting long-term functional outcomes. » This review highlights a case of a 10-year-old boy with iliosacral Ewing sarcoma. Throughout, the review details the collaborative approach integrating orthopaedic oncology, radiation oncology, medical oncology, pathology, radiology, and rehabilitation staff that is critical to successful treatment. »...

CONCLUSIONS: This study integrates tissue-level, single-cell, and exploratory peripheral hematologic evidence to support immune-related alterations in OS. APOE downregulation and altered peripheral neutrophil-related indices may represent clinically relevant features associated with immune dysregulation in osteosarcoma.

CONCLUSION: The epigenetic signature of BSNS is usually preserved in cases that underwent HGRT, even after many years from the initial onset of BSNS. However, in rare instances, the HGRT component may display epigenetic divergence over time.

This study aimed to evaluate the efficacy and safety of anlotinib combined with limb salvage therapy in patients with osteosarcoma after chemotherapy failure. A retrospective study was conducted on 46 eligible osteosarcoma patients who failed chemotherapy, admitted to our hospital from October 2021 to December 2022. They were divided into 2 groups: 28 patients in the anlotinib combined with limb salvage surgery group (combined group) and 18 patients in the limb salvage surgery alone group...

A 10-year-old poodle with left maxillofacial osteosarcoma received volumetric modulated arc therapy delivering 32 Gy in four fractions. Treatment planning was guided by computed tomography-magnetic resonance imaging (CT-MRI) fusion for precise target delineation near the globe. Serial CT and MRI up to 12 months after treatment showed transient enlargement of intratumoral cysts with fluid-fluid levels immediately after treatment, indicating pseudoprogression, followed by marked tumor regression....

Lower extremity (LE) reconstruction presents unique challenges due to complex wound environments, compromised vascularity, and high functional demands. Compared with other anatomic regions, free flap reconstruction in the LE demonstrates higher complication and failure rates. Early microsurgical intervention improves outcomes, though negative pressure wound therapy can bridge to delayed coverage. In oncologic patients, radiation and chemotherapy impair tissue quality and recipient vessels,...

INTRODUCTION: Time-to-treatment initiation (TTI) is an increasingly recognized factor influencing survival in cancer care. Although prolonged TTI has been associated with worse survival in various malignancies, its impact on patients with localized extremity non-small-round-cell sarcoma (NSRCS) remains unclear.

Soft-tissue sarcoma (STS) poses unique challenges to treatment due to its rarity and heterogeneity. Comprised of multiple histologies which, among other characteristics, differ in their genetic makeup, tumor microenvironment, recurrence patterns and therefore response to treatment, STS is treated primarily with surgical resection, with systemic therapy used in high-risk or metastatic cases. In the era of immunotherapy, STS has been characterized as predominantly immune-cold with few histologies...

CONCLUSION: A margin greater than or equal to 1 mm showed reduced LR and less metastasis in patients with parosteal osteosarcoma of the distal femur.

CONCLUSION: PA demonstrated robust anti-osteosarcoma activity in vitro and in vivo. Its mechanisms include blocking proliferation, inducing apoptosis and cell cycle arrest, attenuating metastatic behaviors linked to EMT, and modulating autophagy, possibly through interference with the TNF/NF-κB signaling axis. These findings suggest that PA is a promising candidate for osteosarcoma therapy, warranting further preclinical and clinical evaluation.

CONCLUSIONS: Pan-TRK IHC can be considered an initial screening tool to identify sarcomas potentially harbouring NTRK fusions. However, the presence of diffuse pan-TRK immunoreactivity in NTRK-wild-type tumours highlights the need for cautious interpretation of IHC results. FISH may represent a useful intermediate diagnostic tool when NGS is unavailable but requires cautious interpretation particularly in cases with atypical or isolated signals. NGS-based molecular confirmation remains essential...

OPINION STATEMENT: Hemithorax irradiation (HT‑RT) is a cornerstone multimodal treatment for high‑risk chest wall bone and soft tissue sarcomas, particularly Ewing sarcoma in children and young adults. Current non‑randomized evidence consistently shows HT‑RT reduces systemic relapse-especially lung metastases-without compromising local control or causing unmanageable acute toxicities, and modern techniques (IMRT, VMAT, proton therapy) further improve its therapeutic ratio by lowering late...

PURPOSE OF REVIEW: This review aims to assess the current state of bone and soft tissue sarcomas (STS) management in the adolescent and young adult population (AYA) with a focus on Ewing sarcoma (EWS), osteosarcoma (OS), rhabdomyosarcoma (RMS), and non-rhabdomyosarcoma soft tissue sarcoma (NRSTS).

CONCLUSION: A model combining tumor radiomics and clinical parameters at diagnosis showed strong performance in predicting chemoresistant osteosarcoma, with results confirmed by external validation. This approach may support personalized treatment strategies in high-grade osteosarcoma.

Lymphangioma-like Kaposi sarcoma (LLKS) is a rare histologic variant of Kaposi sarcoma that can closely mimic benign vascular proliferations, posing a diagnostic challenge-especially in immunocompetent individuals. We report a case of LLKS in an HIV-negative elderly man who presented with unilateral, sporotrichoid violaceous papules and plaques confined to the upper limb. Histopathologic examination demonstrated irregularly dilated vascular channels with positive immunostaining for human...

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CONCLUSION: This study provided several M2 macrophage-associated factors as the valuable prognostic biomarkers for OS.

This study evaluated the safety, immunogenicity, and post-vaccination immune responses of timely, sequential catch-up vaccination in children with Kaposiform hemangioendothelioma (KHE) undergoing sirolimus treatment. From July 2020 to November 2021, sixteen children with KHE receiving sirolimus in sustained remission were enrolled, along with 15 age-matched healthy children as controls. Patients in the sirolimus-treated group underwent immune assessment prior to receiving catch-up vaccinations,...

CIC::DUX4 sarcomas are aggressive soft tissue tumours that often mimic Ewing sarcoma but are distinct in their molecular and clinical behaviour. We describe a young adult male initially managed for a presumed primitive neuroectodermal tumour, later confirmed to have a CIC::DUX4 fusion-positive sarcoma through fluorescence in situ hybridisation and NGS analysis. Despite initial response to Ewing-like chemotherapy (vincristine, doxorubicin and cyclophosphamide regimen), the disease rapidly...

Undifferentiated pleomorphic sarcoma (UPS) is an aggressive mesenchymal malignancy with a poor prognosis in unresectable head and neck cases. A woman in her late 70s presented with unresectable maxillary UPS (cT4bN0M0, Stage IVB) involving the skull base and showing membranous epidermal growth factor receptor (EGFR) expression in 10%-20% of tumour cells. After a partial response to definitive chemoradiotherapy, she underwent two sessions of Alluminox photoimmunotherapy with Akalux...