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Histological Type-Specific Behavior in Sarcomas: Analysis of Head and Neck Cancer Registry of Japan.
Laryngoscope
January 2025
Department of Otolaryngology, Head and Neck Surgery, The University of Tokyo, Tokyo, Japan.
Objective: The goal of this study was to better understand the epidemiology, clinical characteristics, and treatment outcomes of head and neck sarcomas using real-world data from Japan.
Methods: Using the Japanese Head and Neck Cancer Registry, we identified 438 patients who were pathologically diagnosed with head and neck sarcoma between 2011 and 2020. We compared epidemiological, clinical, and prognostic data for the different histological types of sarcoma.
PAX3-FOXO1, an oncogenic transcription factor, drives a particularly aggressive subtype of rhabdomyosarcoma (RMS) by enforcing gene expression programs that support malignant cell states. Here we show that PAX3-FOXO1 RMS cells exhibit altered pyrimidine metabolism and increased dependence on enzymes involved in pyrimidine synthesis, including dihydrofolate reductase (DHFR). Consequently, PAX3-FOXO1 cells display increased sensitivity to inhibition of DHFR by the chemotherapeutic drug methotrexate, and this dependence is rescued by provision of pyrimidine nucleotides.
View Article and Find Full Text PDF[Infantile rhabdomyofibrosarcoma with EGFR kinase domain duplication: a clinicopathological analysis of three cases].
Zhonghua Bing Li Xue Za Zhi
February 2025
Department of Pathology, Foshan Traditional Chinese Medicine Hospital, Foshan 528000, China.
To investigate the clinicopathological and genetic features of infantile rhabdomyofibrosarcoma (IRFS) with EGFR kinase domain duplication (EGFR-KDD). The clinical, morphological and immunohistochemical features of three IRFS with EGFR-KDD diagnosed from January 2022 to January 2024 at Department of Pathology, Foshan Traditional Chinese Medicine Hospital, Foshan, China were retrospectively analyzed using PCR or next generation sequencing technique; and related literature was reviewed. There were 1 male and 2 females, aged at presentation ranging from 1 to 4 years.
View Article and Find Full Text PDF[Advances in the pathology of soft tissue in China over the past ten years: retrospect and prospect].
Zhonghua Bing Li Xue Za Zhi
February 2025
Department of Pathology, Fudan University Shanghai Cancer Center, Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200032, China.
During the past decate, Chinese pathologists have made remarkable achievements in the area of soft tissue tumors. They have not only done in-depth researches in selected entities like liposarcoma and round cell sarcomas, but have also issued expert consenses and guideline, as well as published professional books and translation books, with purpose to comprehensively improve the level of diagnosis nationwide.
View Article and Find Full Text PDFAlveolar Rhabdomyosarcoma of Nasopharynx and Paranasal Sinuses in Children Diagnosis and Treatment-Review of the Literature and Case Report.
Medicina (Kaunas)
January 2025
ENT Clinic Department, "Grigore T. Popa" University of Medicine and Pharmacy Iasi, Universitatii Street 16, 700115 Iasi, Romania.
Alveolar rhabdomyosarcoma (aRMS) is a rare pediatric malignant tumor with a poor prognosis, particularly when located in the rhinopharynx and sphenoidal floor, which complicates diagnosis and increases the risk of misclassification as benign growths. The specific genotype of aRMS is associated with a worse clinical outcome. In young children, especially those aged 4 to 12 years, rhinopharyngeal masses are often attributed to chronic adenoiditis; however, other benign (e.
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