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
Bladder malignancy in patients with congenital bladder anomalies who have undergone bladder augmentation is a rare but well-recognized condition. These patients present with locally advanced or metastatic disease and have poor survival. We report a case of a patient with myelomeningocele who was incidentally found to have a high-grade intestinal type adenocarcinoma of her bladder augment at the time of cystolithotomy. This case highlights the need to continue to follow patients with congenital bladder anomalies and highlights the lack of adequate screening methods available.