Rhabdomyosarcoma is a very rare malignant tumor of the larynx which can present in a number of histologic variants. The pleomorphic variant of the tumor has been described in 16 patients to date. Being a rarity, rhabdomyosarcoma is as a rule managed according to the principle of treatment for malignant mesenchymal tumors of the head and neck, and includes radical surgery with postoperative radiotherapy and/or chemotherapy. A case is presented of a pleomorphic rhabdomyosarcoma of the vocal cord. Successfully treated by endoscopic CO2 laser cordectomy with adjuvant chemotherapy and 6-year uneventful follow up.
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Sarcomas developed in patients with Lynch Syndrome are enriched in pleomorphic soft-tissue sarcomas and are sensitive to immunotherapy.
Eur J Cancer
December 2024
Department of Digestive Medical Oncology, Toulouse University Hospital, Toulouse, France; Toulouse Cancer Research Centre, Toulouse, France; Department of Oncogenetics, Oncopole Claudius Regaud, Toulouse, France; Groupe Génétique et Cancer, Unicancer, France. Electronic address:
Article Synopsis
- Sarcomas are not typically associated with Lynch Syndrome (LS), but recent literature suggests a connection, prompting a national study to investigate their characteristics in LS patients.
- The SarcLynch study included 81 patients, finding that 83% had soft-tissue sarcomas, particularly pleomorphic variants like undifferentiated pleomorphic sarcoma and pleomorphic rhabdomyosarcoma, with 40% having sarcoma as their first cancer event.
- Results showed a high prevalence of mismatch repair deficiency and promising responses to immune checkpoint inhibitors, suggesting the need for screening and potential immunotherapy for these sarcomas.
Article Synopsis
- Liposarcomas are the most common type of soft tissue sarcoma, primarily found in deep tissues and the retroperitoneum.
- They include various classifications such as atypical lipomatous tumors, well-differentiated liposarcoma (WDL), dedifferentiated liposarcoma (DDL), myxoid liposarcoma, and pleomorphic liposarcoma.
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Malignant Phyllodes Tumor of the Breast With Multiple Cutaneous Metastasis Resembling Pleomorphic Rhabdomyosarcoma.
Am J Dermatopathol
December 2024
Department of Pathology, Rutgers University New Jersey Medical School, Newark, NJ; and.
Malignant phyllodes tumor (PT) of the breast is a rare fibroepithelial neoplasm that shows variegated histomorphology and an aggressive clinical course. Cutaneous metastases are rare. A 68 year old woman presented with a palpable left breast mass identified on a routine breast exam.
View Article and Find Full Text PDF[Primary intracranial DICER1-mutant sarcoma: a clinicopathological analysis of seven cases].
Zhonghua Bing Li Xue Za Zhi
December 2024
Department of Pathology, Sun Yat-sen University Cancer Center, Guangzhou510060, China.
To investigate the clinicopathological features, immunophenotype, molecular characteristics, and differential diagnosis of primary intracranial DICER1-mutant sarcoma in order to better understand this tumor type. A retrospective analysis was conducted on 7 cases of primary intracranial DICER1-mutant sarcoma diagnosed in the Department of Pathology, Sun Yat-sen University Cancer Center, Guangzhou, China between 2021 and 2023 using next-generation sequencing. At the same time, 10 gliosarcomas, 4 intracranial FET::CREB fusion-positive mesenchymal tumors, 4 malignant meningiomas, 3 malignant solitary fibrous tumors, 3 malignant peripheral nerve sheath tumors, 3 synovial sarcomas and 3 rhabdomyosarcomas (total 30 cases) were selected as control.
View Article and Find Full Text PDFDysregulation of miRNAs in Soft Tissue Sarcomas.
Cells
November 2024
Department of Biomedical Sciences, University of Sassari, Viale San Pietro 43/b, 07100 Sassari, Italy.
Article Synopsis
- * The review focuses on the roles of miRNAs in various soft tissue sarcoma subtypes and how altered miRNA activity can act as oncogenes or tumor suppressors, affecting cancer progression.
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