&lt;p&gt;Identification Of Actionable Genetic Targets In Primary Cardiac Sarcomas&lt;/p&gt;

Coloma, Carmen Salvador; Saigí, Maria; Díaz‐Beveridge, Roberto; Penín, R.M.; Foix, Maria Pane; Mayordomo, Émpar; Melian, Marcos; Schuler, Mona; Muro, Xavier García del; Mora, Jaime Font de

doi:10.2147/ott.s214319

Cited by 5 publications

(3 citation statements)

References 30 publications

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“…Primary malignant HT are rare both in human and veterinary medicine. As a result, even in human medicine large-scale epidemiological studies on their occurrence or randomized control trials and meta-analyses on their treatment are lacking [ 32 ]. Moreover, cytological assessment of sarcomas can be challenging.…”

Section: Discussionmentioning

confidence: 99%

A primary multiple pleomorphic rhabdomyosarcoma of the heart in an adult dog

Szaluś-Jordanow,

Czopowicz,

Moroz-Fik

et al. 2023

BMC Vet Res

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Background Heart tumors are rare in dogs. They can be benign or malignant. Clinical signs depend primarily on the location of the tumor and its effect on blood flow. Case presentation An eleven-year-old crossbreed male dog lethargic and anorectic for previous 3 days was presented to the veterinary clinic. The focused ultrasound assessment with sonograms in trauma (FAST) revealed multiple tumors in the heart which were then confirmed in echocardiographic examination performed by a veterinary cardiologist. Due to the poor general condition and grave prognosis, the dog was humanely euthanized. The autopsy revealed numerous intracardiac tumors in all four heart chambers. No proliferative changes were found in other organs either in thoracic or abdominal cavity. Immunohistochemical examination was performed using formalin-fixed, paraffin-embedded tissue from heart masses. The antibodies against myoglobin, desmin, smooth muscle actin, vimentin, CD34, S100, and pan-cytokeratin (AE1/AE3) were used. Microscopically, the tumor was composed of fascicles of spindle-shaped cells with pale eosinophilic cytoplasm with round, oval, and focally elongated nuclei and one or two prominent nucleoli. The tumor cells showed strong diffuse cytoplasmic immunopositivity for myoglobin and vimentin and focal staining for desmin. Immunostainings for smooth muscle actin-SMA, CD34, pan-cytokeratin, S-100 protein were negative. The immunohistochemical staining pattern confirmed rhabdomyosarcoma. Conclusions This is the first description of the primary multiple heart rhabdomyosarcoma in a dog.

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Section: Discussionmentioning

confidence: 99%

A primary multiple pleomorphic rhabdomyosarcoma of the heart in an adult dog

Szaluś-Jordanow,

Czopowicz,

Moroz-Fik

et al. 2023

BMC Vet Res

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“…Indeed, the classification of UHGPC-SRCSs requires immunohistochemistry, and vimentin expression is considered reliable for this type of pSTS-h [8], as well as αsmooth muscle actin and cytokeratins [8]. In addition, chromosomal defects of AKT2 and RUNX2, mutation of PDGFRB, and a higher number of chromosomal aberrations have been described in these rare variants of SRCs [26].…”

Section: Undifferentiated High-grade Pleomorphic Sarcomamentioning

confidence: 99%

Primary Soft Tissue Sarcoma of the Heart: An Emerging Chapter in Cardio-Oncology

Scicchitano¹,

Sergi

Cameli

et al. 2021

Biomedicines

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Primary malignant cardiac tumors are rare, with a prevalence of about 0.01% among all cancer histotypes. At least 60% of them are primary soft tissue sarcomas of the heart (pSTS-h) that represent almost 1% of all STSs. The cardiac site of origin is the best way to classify pSTS-h as it is directly linked to the surgical approach for cancer removal. Indeed, histological differentiation should integrate the classification to provide insights into prognosis and survival expectancy of the patients. The prognosis of pSTS-h is severe and mostly influenced by the primary localization of the tumor, the difficulty in achieving complete surgical and pharmacological eradication, and the aggressive biological features of malignant cells. This review aims to provide a detailed literature overview of the most relevant issues on primary soft tissue sarcoma of the heart and highlight potential diagnostic and therapeutic future perspectives.

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“…Immune checkpoint inhibitor (ICI)-based immunotherapy has shown promising efficacy in various malignancies ( 7 – 9 ). Evidence regarding ICI efficacy in RMS remains scarce ( 10 ). A multicenter phase 2 clinical trial showed that ICIs exhibited promising efficacy and an acceptable safety profile in advanced STS ( 11 ).…”

Section: Introductionmentioning

confidence: 99%

Case Report: A PD-L1-Positive Patient With Pleomorphic Rhabdomyosarcoma Achieving an Impressive Response to Immunotherapy

Liu

Gong³

et al. 2022

Front. Immunol.

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There is currently a lack of effective systemic treatment for patients with advanced pleomorphic rhabdomyosarcoma (PRMS). Although programmed death protein 1 (PD-1) inhibitors have shown efficacy in various solid tumors, their effects on PRMS have not been well established. Here, we present a case of a 12-year-old Chinese male adolescent with metastatic PRMS who benefited from the PD-1 inhibitor nivolumab. The patient initially underwent primary tumor resection but failed to respond to subsequent first-line chemotherapy and second-line pazopanib treatment. Pathological examination showed positive PD-L1 expression and tumor-infiltrating lymphocytes in the tumor tissue, and the patient was administered nivolumab as a posterior-line treatment. After attaining a clinically partial response (PR), surgical resection was performed, which was followed by adjuvant nivolumab. At the time of the submission of this manuscript, the patient achieved recurrence-free survival (RFS) lasting 45 months and counting. This is the first clinical evidence that a patient with refractory PRMS was controlled by anti-PD-1 antibody, with an RFS lasting more than 3 years. This case suggests that PD-L1 expression and T-cell infiltration could be used as potential biomarkers for PRMS immunotherapy.

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<p>Identification Of Actionable Genetic Targets In Primary Cardiac Sarcomas</p>

Cited by 5 publications

References 30 publications

A primary multiple pleomorphic rhabdomyosarcoma of the heart in an adult dog

A primary multiple pleomorphic rhabdomyosarcoma of the heart in an adult dog

Primary Soft Tissue Sarcoma of the Heart: An Emerging Chapter in Cardio-Oncology

Case Report: A PD-L1-Positive Patient With Pleomorphic Rhabdomyosarcoma Achieving an Impressive Response to Immunotherapy

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