Primary Renal Ewing Sarcoma in Children and Young Adults
| Autor: | Alexander Nobori, Wendy Allen-Rhoades, Noah Federman, Moran Gotesman, Bindi Naik-Mathuria, Jerry Cheng, Jeffrey Goldstein, Eduard H. Panosyan, Alan Ikeda, Kathryn L. Bradford, Arun S. Singh, Brittany L. Johnson, Joseph L. Lasky |
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| Rok vydání: | 2020 |
| Předmět: |
Male
Oncology Kidney Disease pediatric malignancy Disease Cardiorespiratory Medicine and Haematology Metastasis 0302 clinical medicine Young adult Medical diagnosis Child Cancer Pediatric Kidney education.field_of_study Soft tissue Sarcoma Hematology Kidney Neoplasms medicine.anatomical_structure small round blue cell tumors 030220 oncology & carcinogenesis Female primary renal Ewing sarcoma Pediatric Research Initiative medicine.medical_specialty Adolescent Pediatric Cancer Population Renal and urogenital Sarcoma Ewing Article 03 medical and health sciences Rare Diseases Clinical Research Ewing Internal medicine medicine Humans Oncology & Carcinogenesis education business.industry medicine.disease Pediatrics Perinatology and Child Health business Ewing sarcoma 030215 immunology |
| Zdroj: | Journal of pediatric hematology/oncology, vol 42, iss 8 J Pediatr Hematol Oncol |
| ISSN: | 1077-4114 |
| Popis: | The Ewing sarcoma family of tumors (ESFT) are high-grade small round blue cell malignancies traditionally presenting in children and adolescents. The most common site of primary disease is bone, though extraskeletal primary sites are well-recognized.() We present six cases of primary ESFT of the kidney and one case of the adrenal gland. Patients were 11–18 years of age at diagnosis. Metastases at diagnosis were present in most cases (n=6). All patients underwent surgery, and most received radiation (n=5). Five patients relapsed after initial remission. Comprehensive review of the primary renal ESFT literature was used to analyze various factors, including age, gender, disease metrics, metastases at diagnoses, and overall survival in a total of 362 cases. Notably, while the general ESFT population has reported rates of metastasis at diagnosis of 20–25%,(2) this rate in the renal ESFT population was 53% with a rate of 59% in adolescent and young-adult (AYA) patients (11–24 years). Nodal disease at diagnosis was present in 24% of renal ESFT cases compared with 3.2% in patients with primary skeletal ESFT.(3) While this malignant process may share histologic and molecular features with its bone and soft tissue counterparts, primary renal ESFT presentations appear to be more aggressive and have worse outcomes. |
| Databáze: | OpenAIRE |
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