☆ 4.7 Article

Characteristics and Outcomes in Pediatric Non-Central Nervous System Malignant Rhabdoid Tumors: A Report from the National Cancer Database

ANNALS OF SURGICAL ONCOLOGY (2021)

Journal

ANNALS OF SURGICAL ONCOLOGY

Volume -, Issue -, Pages -

Publisher

SPRINGER

DOI: 10.1245/s10434-021-10370-x

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Abstract

Pediatric non-CNS MRTs are rare and aggressive malignancies. Age <1 year and presence of metastasis are negative prognostic indicators, while surgical intervention can improve survival rates. However, the presence of residual disease may lead to worse outcomes.

Background Pediatric non-central nervous system (CNS) malignant rhabdoid tumors (MRTs) are rare and aggressive malignancies without standard treatment strategies. The National Cancer Database (NCDB) was utilized to describe the incidence, characteristics, treatment strategies, and outcomes in pediatric patients. Methods Patients Results Overall, 202 patients were identified. Soft tissue tumors were most common and metastatic disease was present at diagnosis in 34.2% of patients. The 1- and 5-year overall survival (OS) rates were 48.8% and 35.9%, respectively. Multivariable analysis revealed that age <1 year and the presence of metastasis were negative prognostic indicators (p = 0.058). The 1- and 5-year OS rates were 59.9% and 46.5%, respectively, for patients who received surgical intervention (n = 143) compared with 12.3% and 7.4%, respectively, for those treated nonoperatively (n = 59; p < 0.01). Surgical resection was associated with improved outcomes on univariate analysis, although it was no longer an independent predictor of survival on multivariate analysis (p = 0.18). In the cohort of surgical patients, the presence of residual disease trended towards clinically significant worse outcomes (p = 0.13). Conclusions Patients with non-CNS MRTs who were diagnosed in infancy and had metastatic disease had worse survival outcomes. Although surgical resection was associated with improved survival in non-CNS MRTs, it was not independently associated with survival on multivariate analysis. Efforts to improve survival may instead depend on improving chemotherapeutic strategies and developing targeted therapies.

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Outcomes of Octogenarians with Primary Malignant Cardiac Tumors: National Cancer Database Analysis

Mohamed Rahouma, Massimo Baudo, Anas Dabsha, Arnaldo Dimagli, Abdelrahman Mohamed, Stephanie L. Mick, Leonard Girardi, Mario Gaudino, Roberto Lorusso

Summary: This study analyzed mortality differences among different age groups of patients with PMCTs and found that with increasing age, patients had a higher comorbidity burden, received more conservative treatment, and experienced progressively declining early and late survival outcomes.

JOURNAL OF CLINICAL MEDICINE (2022)