Factors predicting surgical receipt in extracranial rhabdoid tumors.

Abstract

e23541 Background: Extracranial rhabdoid tumors (ERTs) are a very rare and aggressive carcinoma that grow in a wide range of tissues, including but not limited to the lungs, kidneys, muscles, skin, and other soft tissues. These tumors most commonly present in infants between 11-18 months old, with an estimated annual incidence of less than 1 per million in the United States. Metastasis is common, and average survival time after diagnosis is approximately 5 years. Though most patients appear to undergo tumor resection at the primary site, a standardized treatment protocol for ERTs has not yet been developed. This study aimed to analyze the factors predicting receipt of this surgery. Methods: The National Cancer Database (NCDB) was used to identify patients diagnosed with SEF from 2004 to 2020 using histology code 8963 as assigned by the Commission on Cancer Accreditation program. Kaplan-Meier, ANOVA Chi-Square, and Logistic Regression tests were performed, and data were analyzed using SPSS version 29. Statistical significance was set at α = 0.05. Results: 208 patients with extracranial rhabdoid tumors formed the initial sample. Of these patients, 128 patients (61.5%) received surgery at the primary site. 80 patients (38.5%) did not have tumor resection. Median overall survival of surgical patients was significantly higher than nonsurgical patients ( 91.6 months vs. 32.1 months, p < 0.001). Head, neck, and face, were the most common primary tumor sites (25.4%). Wider surgical margins were associated with improved outcomes ( p < 0.05). On nominal regression, stage I, II, and III disease, grade 1 and 2, and younger age were associated with increased likelihood of receiving surgery ( p < 0.05). Metastasis to distant sites was associated with decreased likelihood of receiving surgery ( p < 0.05). Treatment at an academic facility, income status, tumor size, insurance status, and race were not significant predictors of receiving surgery. 77 patients (37%) received adjuvant chemotherapy, 1 patient received adjuvant radiation, and 4 patients received adjuvant chemoradiation. 121 patients (58.2%) did not receive adjuvant therapy. Conclusions: Resection of the primary tumor in ERT is associated with improved overall survival. Younger age, stage I, II, or III disease, and grade 1 or 2 disease are factors predicting a higher likelihood of receiving surgery. Improved outcomes of tumor resection are associated with wider surgical margins. The presence of metastasis decreased likelihood of undergoing surgery. Treatment location, insurance, status, race, income, and tumor size did not affect surgery receipt. Interestingly, the majority of patients sampled did not receive any adjuvant therapies, such as chemotherapy or radiation. Future research should continue delineating what factors predict both receipt of surgery and improved surgical outcomes, as well as better understand what the role of adjuvant therapies is in managing ERT.