RESUMO
OBJECTIVE: To identify factors impacting overall survival (OS) in children, adolescents, and young adults with high-risk renal tumors (HRRTs). METHODS: The National Cancer Database was queried for patients ≤30 years old diagnosed with anaplastic Wilms tumors (AWT), clear cell sarcoma of the kidney (CCSK), or rhabdoid tumor of the kidney (RTK) between 2004 and 2013. Demographic, clinical, and OS data were abstracted. OS between groups was compared with a Kaplan-Meier curve. Univariate and multivariate survival analyses were performed. RESULTS: A total of 349 patients were identified meeting criteria; 133 (38.1%) AWT, 120 (34.4%) CCSK, and 96 (27.5%) RTK. Patients with RTK were less likely to undergo surgery than those with AWT or CCSK (77.1% vs 94% vs 99%, P < .001) and less likely to receive chemotherapy (84.4% vs 96.2% vs 95%, P = .013) or radiation (52.1% vs 81.2% vs 86.7%, P < .001). Estimated 5-year OS was 76.1% (95% confidence interval [CI] 67.9-84.4) for AWT, 92.7% (95% CI 87.4-97.9) for CCSK, and 33.5% (95% CI 23.1-43.9) for RTK (P < .001). On multivariate analysis, AWT (HR 3.372, P = .032) and RTK histology (HR 12.595, P < .001) were significantly associated with worse OS, while receiving radiation (HR 0.43, P = .006) was associated with improved OS. LN positivity, margin status, and undergoing surgery were not. Analyzing the HRRTs individually, for AWT, undergoing surgery was associated with OS (HR 0.308, P = .031). For RTK, factors associated with OS included undergoing surgery (HR 0.209, P = .007) and radiation (HR 0.411, P = .008). CONCLUSION: Within the HRRTs, RTK is associated with worse outcomes than either AWT or CCSK. Receiving radiation is significantly associated with improved outcomes, and surgery is important for those with AWT and RTK.
Assuntos
Neoplasias Renais/mortalidade , Tumor Rabdoide/mortalidade , Sarcoma de Células Claras/mortalidade , Tumor de Wilms/mortalidade , Adolescente , Adulto , Antineoplásicos/uso terapêutico , Criança , Pré-Escolar , Bases de Dados Factuais , Uso de Medicamentos , Feminino , Humanos , Lactente , Recém-Nascido , Neoplasias Renais/patologia , Neoplasias Renais/terapia , Metástase Linfática , Masculino , Análise Multivariada , Nefrectomia/estatística & dados numéricos , Radioterapia Adjuvante , Tumor Rabdoide/patologia , Tumor Rabdoide/terapia , Fatores de Risco , Sarcoma de Células Claras/patologia , Sarcoma de Células Claras/terapia , Estados Unidos/epidemiologia , Tumor de Wilms/patologia , Tumor de Wilms/terapia , Adulto JovemRESUMO
OBJECTIVE: The purpose of this study is to present our experience with ten cases of Central nervous system atypical teratoid rhabdoid tumor (CNS/ATRT). PATIENTS AND METHODS: A series of ten patients with CNS/ATRT, were diagnosed and treated between 1990 and 2005, at the National Institute of Pediatrics, in Mexico City. The gender, age of presentation, clinical features, tumor localization, imaging studies, grade of tumor resection, complications, adjuvant therapy, and survival are presented. RESULTS: The mean age at diagnosis was 37.8 months, seven cases were male, and their average clinical course was 1.3 months. The more common clinical presentation was intracranial hypertension with cranial nerve deficits; location was infratentorial in four patients and supratentorial in six. Hydrocephalus was present as the most common complication (seven cases). In nine patients, the grade of resection was total or subtotal. In one case, it was only possible to perform a biopsy. There were two cases with longer survival (9 and 16 months), and their tumors were resected in total or subtotal manner and received adjuvant therapy (radiotherapy and chemotherapy). CONCLUSIONS: Preliminary results, show that in older children, we can improve their survival with the subtotal or total resection of the tumor and the addition of chemotherapy and radiotherapy.