Renal sarcomas in children and adolescents: a retrospective, multicenter cohort study
Notice bibliographique
Résumé
Background: Renal sarcomas arise rarely in children and adolescents and represent a histologically and biologically diverse disease category. Consequently, standardizing optimal therapies for pediatric renal sarcomas remains challenging. Leveraging a large North American research collaborative, the purposes of this study were to evaluate the current state of patient, disease, and survival characteristics among pediatric renal sarcomas and to expose knowledge gaps that will inform future discovery. Methods: Patients 21 years or younger and treated for a primary renal sarcoma between January 1st, 2000 and November 30th, 2022 were identified through the Pediatric Surgical Oncology Research Collaborative. Patient (e.g., demographics) and disease (e.g., histology, stage, molecular alterations) characteristics were abstracted from contributing institutions. Descriptive statistics, Pearson-Chi square (categorical variables), Kruskal-Wallis (continuous variables), Cox regression (Hazard ratios), and Kaplan-Meier 4-year event-free and overall survival (OS) analyses were completed. Findings: Among 158 patients, clear cell sarcoma of the kidney (CCSK; n = 94), Ewing sarcoma (EWS; n = 33), and undifferentiated sarcoma (n = 8) predominated. Sarcoma type correlated significantly with age at diagnosis (p < 0.0001), with infantile fibrosarcoma (IFS) and CCSK occurring in the youngest patients, whereas EWS and synovial sarcoma presented in the oldest. Predisposition syndromes were identified in 11/155 (7.1%) patients, most commonly DICER1 and Li-Fraumeni. Multimodal therapies varied significantly across sarcoma types (p = 0.0008), although nephrectomy was uniform. Tumor thrombectomy was performed in 9 patients (6 with EWS). When tested, somatic molecular alterations were observed principally in CCSK (17/38; 45%) and EWS (26/26; 100%; p = 0.001). At 4 years, OS differed significantly by sarcoma type, ranging from highest to lowest as follows: CCSK 0.927 (95% CI 0.845-0.967), EWS 0.901 (95% CI 0.723-0.967), undifferentiated sarcoma 0.833 (95% CI 0.273-0.975), IFS 0.667 (95% CI 0.054-0.945), and rhabdomyosarcoma 0.500 (95% CI 0.111-0.804; p = 0.036). Hematogenous metastases occurred most in the lungs (n = 19 total; 10 with EWS), followed by bone (n = 12), which occurred only with CCSK (n = 9) and EWS (n = 3). Two patients developed brain metastases (one each with CCSK and rhabdomyosarcoma). At 4 years, OS was 0.957 (95% CI 0.888-0.984) for patients presenting without metastases and 0.717 (95% CI 0.545-0.833) for those with metastases (p = 0.00015). Interpretation: Renal sarcomas presenting in children and adolescents comprise a heterogeneous disease category with unique patient, clinical, and molecular characteristics that complicate standardizing therapeutic strategies beyond CCSK and EWS. Funding: None.
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Comment cette classification a été obtenuedéplier
Prédiction distillée sur la base complète
Imitation des enseignantsNi prévalence calibrée, ni vérité terrain. Validation humaine à venir. Apprise à partir de 10 348 étiquettes directes de Codex et de 10 348 étiquettes directes de Gemma. Le mode candidate est l'union des têtes enseignantes seuillées; le consensus est leur intersection. Ces sorties portent le statut machine_predicted_unvalidated et ne sont ni des étiquettes humaines ni des étiquettes directes de modèles de pointe.
Scores Codex et Gemma par catégorie
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,000 | 0,000 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,000 | 0,000 |
| Bibliométrie | 0,000 | 0,000 |
| Études des sciences et des technologies | 0,000 | 0,000 |
| Communication savante | 0,000 | 0,000 |
| Science ouverte | 0,000 | 0,000 |
| Intégrité de la recherche | 0,000 | 0,000 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,000 | 0,000 |
Scores machine (provisoires)
Les deux têtes enseignantes du modèle étudiant, lues sur ce travail. Un score ordonne la base pour la relecture; il n'affirme jamais une catégorie, et le statut de validation accompagne chaque rangée tel quel.
Scores de référence d'un modèle non mature (critères de maturité non atteints, 7 itérations). Un score ordonne; il n'affirme jamais une catégorie.
score_only:v0-immature-baseline · tel quel depuis la passe de notation : score_only signifie que le nombre peut ordonner les travaux, et qu'aucune étiquette de catégorie n'en découleClassification
machine, non validéePrédiction automatique; un appel candidat d’une seule tête enseignante, pas un consensus.
Le détail, modèle par modèle et score par score, se trouve en fin de page sous « Comment cette classification a été obtenue ».