IMG-22. ASL PERFUSION LIGHTBULB SIGN: A POTENTIAL PATHOGNOMONIC FEATURE OF PEDIATRIC POSTERIOR FOSSA HEMANGIOBLASTOMA

Abstract

Abstract INTRODUCTION Hemangioblastoma is a rare vascular tumor which occurs within the central nervous system in children. Differentiating hemangioblastoma from other posterior fossa tumors can be challenging on imaging and preoperative diagnosis can change neurosurgical approach. We hypothesize a ‘lightbulb sign’ (diffuse homogenous intense hyperperfusion within solid component of tumor) will help differentiate hemangioblastoma from other posterior fossa tumors. METHODS In this retrospective case-control study, we only included pathology-proven cases of hemangioblastoma, while the control group consisted of other randomly selected pathology-proven posterior fossa tumors from January 2022 to January 2024. Two blinded neuroradiologists analyzed all applicable MRI sequences. Their perfusion analysis also included the “lightbulb sign” as an extra criterion. Disagreements between the radiologists were confirmed by a third pediatric neuroradiologist. Chi-square and Fisher’s exact test were used to analyze the data. RESULTS 95 patients were enrolled in the study, 57(60%) were male. The median age at diagnosis was 8 (IQR: 3-14). Out of the enrolled patients, 8 had hemangioblastoma, and 87 had other posterior fossa tumors, including medulloblastoma (n=31), pilocytic astrocytoma (n=23), posterior fossa ependymoma type A (n=16), and other tumors (n=17). The comparison of hemangioblastoma vs non-hemangioblastoma showed that peripheral edema (p=0.020, OR=1.8) and T2 flow void (p=0.021, OR=1.7) favors hemangioblastoma, whereas reduced diffusion (low ADC) (p=0.002, OR=2.3) and ventricular system extension (p=0.001, OR=2.4) favors non-hemangioblastoma tumors. 42 cases also had perfusion studies. While high perfusion favors hemangioblastoma (p=0.021, OR=2.9), the lightbulb sign shows a complete distinction since all the ASL series of hemangioblastoma cases (n=4) showed the lightbulb sign, whereas none of the non-hemangioblastoma cases (n=38) showed the sign (p<0.001). CONCLUSION Lightbulb-like intense and homogenous hyperperfusion pattern on ASL allows differentiation of hemangioblastoma from other posterior fossa tumors in children.