Validating the UPMC JNA Staging System: 10-Year Experience from a Tertiary Pediatric Hospital

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Introduction: Juvenile nasopharyngeal angiofibromas (JNAs) are benign but highly vascular, locally aggressive skull base tumors with a male predisposition. Management of larger, more extensive JNAs carries high perioperative risks and morbidity, requiring the expertise of a multidisciplinary skull base team for treatment. Various staging systems have been published to classify these tumors based on size, extent of disease, or perioperative morbidity. The UPMC staging system is unique in that it uses residual vascularity and intracranial extension to predict blood loss and recurrence when using a primarily endoscopic approach. Reports of clinical use of this system are limited in the current literature. Identifying a comprehensive staging system that assists in treatment planning and patient counseling regarding perioperative risk and postoperative expectations is key to establishing a standard of care. We present our institution’s JNA experience in an effort to validate the UPMC staging system as a universal clinically applicable one. Methods: An IRB-approved single institution retrospective study of pediatric patients (<18 years of age) who were diagnosed with JNA were reviewed between January 2011 to September 2023. Patients were excluded if they had incomplete angiographic or operative details, above 18 years of age, refusal to undergo surgical intervention, or without available imaging. Basic patient demographics and preoperative imaging were reviewed to establish the extent of tumor invasion with subsequent application of the UPMC staging system to categorize these tumors. Preoperative angiography and embolization data was reviewed to assess residual vascularity. Intraoperative records were reviewed for total blood loss and staged surgeries. JNA recurrence after complete resection was classified as tumor regrowth. Statistical analysis was performed using SAS v 9.4; SAS Institute Inc., Cary, NC, 2019) and a p -value of less than 0.05 was considered statistically significant. Results: A total of 26 pediatric patients were included in the study, with a mean age of 15.1 years. All 26 of our patients were male. According to the UPMC staging system, 2 were classified as Stage I, 4 as Stage II, and 4 as Stage III, 9 as stage IV, and 7 as stage V. The mean blood loss was 850 mL, and the recurrence rate was 19.2%. The UPMC staging system showed a receiver operating characteristic (ROC) value of 0.75 (95% CI: 0.57–0.93) for predicting the need for staged resection, indicating a fairly good predictive ability. However, for predicting recurrence, the ROC value was 0.53 (95% CI: 0.27–0.79), suggesting limited effectiveness. Conclusion: The UPMC staging system appears to be a reliable tool for predicting the need for staged resection of JNAs, with a ROC value of 0.75. However, its ability to predict recurrence is less certain. Further studies are needed to improve the predictive accuracy for recurrence and to narrow the confidence intervals for both outcomes. Publication History Article published online: 05 February 2024 © 2024. Thieme. All rights reserved. Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany

Ce résumé expose les affirmations des auteurs. BNTIC ne l’interprète pas comme une validation indépendante des résultats.