Pleomorphic rhabdomyosarcoma (RMS) is a rare malignancy, and treatment protocols for this tumor are not established. Although Intergroup Rhabdomyosarcoma Study data have led to improvement in overall and disease-free survival for children with juvenile RMS, the applicability of those findings to adult pleomorphic RMS is uncertain. This report presents the case of a 29-year-old man with pleomorphic RMS of the forearm (a location not previously reported in current literature); reviews the immunohistochemical, ultrastructural, and histologic diagnostic criteria; and discusses treatment.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1053/jhsu.2002.29481 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
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.
Characterization of a pleomorphic rhabdomyosarcoma cell line.
Sci Rep
January 2025
Institute of Pharmacology, Medical University of Vienna, Vienna, Austria.
Pleomorphic rhabdomyosarcoma (PRMS) is an extremely rare soft tissue tumor with dismal prognosis that has a higher incidence in adults compared to the other RMS subtypes. The unique PRMS cell line BH1522 was established from a pleural effusion of a lung metastasis and the characteristics of this cell line were compared to two embryonal type RMS (ERMS) cell lines. The affected patient had been treated by surgery, several cycles of chemotherapy and thoracoscopy of the lung metastases.
View Article and Find Full Text PDFSarcomas 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.
- DDL is especially common in the retroperitoneum and can have both well-differentiated lipomatous and dedifferentiated components, sometimes resembling other aggressive tumors, and can even differentiate into several other cancer types or form bone tissue.
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 PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!