Tumor-derived G-CSF induces an immunosuppressive microenvironment in an osteosarcoma model, reducing response to CAR.GD2 T-cells.

Sarcomas are rare, mesenchymal tumors, representing about 10-15% of all childhood cancers. GD2 is a suitable target for chimeric antigen receptor (CAR) T-cell therapy due to its overexpression in several solid tumors. In this preclinical study, we investigated the potential use of iCasp9.

Complete Clinical Recovery Following Surgical Excision of Extensive Primary Cardiac Sarcoma.

Primary cardiac tumors are rare, most of them being benign. Most malignant cardiac tumors are sarcomas, with the most important therapeutic option being surgery, along with perioperative chemo- and radiotherapy. Here, we present a case of a 67-year-old female patient with no prior medical condition, who presented with primary cardiac sarcoma and extensive tumor growth in the pulmonary artery.

Right Ventricular Metastasis of Ewing Sarcoma Treated Through Surgical Resection.

A 27-year-old man diagnosed with right ventricular metastasis of Ewing sarcoma was referred to our institution. We surgically resected the metastatic tumor to prevent sudden death and reconstructed the right ventricle and tricuspid valve. He has survived for 1 year postoperatively without recurrence.

Intimal sarcoma of the aortic valve and ascending aorta: a case report.

Background: Intimal sarcomas are rare tumors that typically affect the major vessels, such as the pulmonary artery and aorta, and are associated with a particularly poor prognosis. Intimal sarcomas found in the aorta are most commonly located in the abdominal section between the celiac artery and the iliac bifurcation. The descending aorta is involved in 30% of cases, while involvement of the ascending aorta is rare.

Pulmonary endarterectomy through inverted-T upper hemisternotomy.

Objective: We aimed to explore the feasibility of an inverted-T upper hemisternotomy approach for pulmonary endarterectomy (PEA) and report the results after 17 cases.

Methods: PEA was conducted through a 7-cm skin incision using an inverted-T upper hemisternotomy across the third intercostal spaces. Cardiopulmonary bypass (CPB) was established through central arterial and percutaneous femoral dual-staged venous cannulation.

A Phase 1 Study of FHD-609, a Heterobifunctional Degrader of Bromodomain-Containing Protein 9, in Patients With Advanced Synovial Sarcoma or SMARCB1-Deficient Tumors.

Purpose: FHD-609, a potent, selective, heterobifunctional degrader of bromodomain-containing protein 9 (BRD9), was evaluated for treatment of patients with advanced synovial sarcoma (SS) or SMARCB1-deficient tumors.

Patients And Methods: In this multinational, open-label, phase 1 study (NCT04965753), patients received FHD609 intravenously at escalating doses either twice weekly (BIW) (5 to 80 mg; n=40) or once weekly (QW) (40 to 120 mg; n=15).

Results: Fifty-five patients received FHD-609 for a median of 43 days.

Investigating intra-tumoural heterogeneity and microenvironment diversity in primary cardiac angiosarcoma through single-cell RNA sequencing.

Background: Primary cardiac angiosarcoma (PCAS) is a rare and aggressive heart tumour with limited treatment options and a poor prognosis. Understanding cellular heterogeneity and tumour microenvironment (TME) is crucial for the development of effective therapies. Here, we investigated the intratumoural heterogeneity and TME diversity of PCAS using single-cell RNA sequencing (scRNA-seq).

Myeloid sarcoma of the heart-A clinicopathological correlation.

A 63-year-old woman with a history of acute myeloid leukemia followed by stem cell transplantation presented with acute heart failure. Transthoracic echocardiography revealed a preserved left ventricular ejection fraction with severe ventricular hypertrophy and signs of elevated filling pressures indicating infiltrative cardiomyopathy. She died from cardiac arrest due to cardiogenic shock.

tp53 R217H and R242H mutant zebrafish exhibit dysfunctional p53 hallmarks and recapitulate Li-Fraumeni syndrome phenotypes.

Li-Fraumeni syndrome (LFS) is a hereditary cancer predisposition syndrome associated with a highly penetrant cancer spectrum characterized by germline TP53 mutations. We characterized the first LFS zebrafish hotspot mutants, tp53 R217H and R242H (human R248H and R273H), and found these mutants exhibit partial-to-no activation of p53 target genes, have defective cell-cycle checkpoints, and display partial-to-full resistance to apoptosis, although the R217H mutation has hypomorphic characteristics. Spontaneous tumor development histologically resembling human sarcomas was observed as early as 6 months.

Glutamine is critical for the maintenance of type 1 conventional dendritic cells in normal tissue and the tumor microenvironment.

Proliferating tumor cells take up glutamine for anabolic processes, engendering glutamine deficiency in the tumor microenvironment. How this might impact immune cells is not well understood. Using multiple mouse models of soft tissue sarcomas, glutamine antagonists, as well as genetic and pharmacological inhibition of glutamine utilization, we found that the number and frequency of conventional dendritic cells (cDCs) is dependent on microenvironmental glutamine levels.

A Comprehensive Insight Into Primary Intimal Sarcoma of the Pulmonary Artery; From Diagnosis to Management: A Case Report and Review of the Literature.

Primary intimal sarcoma of the pulmonary artery is a rare and aggressive malignancy that presents significant diagnostic and therapeutic challenges due to its nonspecific symptoms and propensity for late detection. This case report aimed to elucidate the diagnostic journey, surgical intervention, and multidisciplinary management of this rare entity. In September 2023, a 42-year-old male presented with dyspnea on exertion and retrosternal chest pain, classified as NYHA FC II.

The Critical Role of Stereotactic Body Radiation Therapy in Multimodal Treatment of Lung Metastasis from Bone and Soft Tissue Sarcomas.

Background: Stereotactic body radiotherapy (SBRT) is increasingly used to treat lung metastasis (LM) in patients with soft tissue sarcoma (STS) and bone sarcoma (BS).

Methods: This retrospective study evaluated the outcomes of patients with BS and STS treated with SBRT for LM between 2010 and 2023.

Results: We enrolled 102 patients (51 each with STS and BS), of whom 71 were males and 31 were females (median age, 40 years; range, 11-81 years).

FUS/circZEB1/miR-128-3p/LBH feedback loop contributes to the malignant phenotype of GSCs via TNF-α-mediated NF-κB signaling pathway.

Glioblastoma (GBM) is the most lethal and common primary tumor of central nervous system with a poor prognosis. Glioma stem cells (GSCs) are particularly significant in GBM proliferation, invasion, self-renewal and recurrence. Circular RNAs (circRNAs) play important roles in various physiological and pathological processes, including regulating the biological behavior of GBM.

Pulmonary Artery Sarcoma with Extensive Invasion of the Right Ventricle: A Case Report and Review of Therapeutic Options.

Pulmonary artery sarcoma (PAS) is a rare, aggressive cancer originating from the intimal layer of the pulmonary artery (PA), often mistaken for pulmonary thromboembolism. This case report underscores the complex management of PAS and the necessity of a multidisciplinary approach for accurate diagnosis and treatment. A 52-year-old woman with PAS was diagnosed using imaging and therapeutic tests to distinguish it from pulmonary embolism.

PDGFRB promotes dedifferentiation and pulmonary metastasis through rearrangement of cytoskeleton under hypoxic microenvironment in osteosarcoma.

Background: Osteosarcoma (OS) cells commonly suffer from hypoxia and dedifferentiation, resulting in poor prognosis. We plan to identify the role of hypoxia on dedifferentiation and the associated cellular signaling.

Methods: We performed sphere formation assays and determined spheroid cells as dedifferentiated cells by detecting stem cell-like markers.

Breast tumor with giant borderline phyllodes: Case report and literature review.

Rationale: Giant phyllodes tumors are rare fibroepithelial neoplasms, accounting for less than 1% of all primary breast tumors. Their main features are a single-round mass, progressive enlargement, and a high rate of local recurrence. A phyllodes tumor measuring more than 10 cm in diameter is usually defined as a "giant" tumor.