Good afternoon everyone. My name is Mohammad Vouda. I'm currently a postdoc fellow at the Department of Neurosurgery at Johns Hopkins University and today I'm going to present one of my projects that I have done before during my time at Boston Children's. Given the fact that the vast majority of the recurrent craniopharyngioma cases in the pediatric population are asymptomatic and clinically stable, it's crucial to know the appropriate timing of intervention and to avoid the burden of any further unnecessary tumor-directed therapies or interventions. In this study we proposed a novel algorithm for the management of pediatric craniopharyngioma at time of recurrence or progression. This is a retrospective core study in which we included all patients less than 21 years old, pre-operative CD brain, pre- and post-operative MRI brain with and without contrast were available for all patients. Recurrence was defined as a newly identified craniopharyngioma following gross tumor resection. Progression was defined as progressive increase in the size of the residual tumor. 80 patients met the inclusion criteria. The median age at time of initial diagnosis was about nine years old and all patients were followed for at least one year with a median follow-up period of 11 years. In order to determine the independent risk factors for tumor recurrence or progression, we ran the univariate and multivariable logistic regression analysis and these turned out to be fine calcification and subtotal resection alone without any adjuvant radiotherapy. In the Kaplan-Meier curves we can see that the subtotal resection only without any adjuvant radiotherapy is associated with the highest risk of recurrence. We can also see that the recurrent cases clustered in the first two years post-operatively. There was also no statistically significant difference between gross tumor resection and subtotal resection with adjuvant radiotherapy in term of tumor control. In this model we have shown the effect of different management strategies on tumors with fine versus coarse calcification. In tumors with fine calcifications, subtotal resection alone is associated with almost 94% risk of recurrence. However, adding radiotherapy dramatically decreased the risk of recurrence to almost 20%. 30 patients have experienced a tumor recurrence or progression. 85% of them were clinically stable. Almost two thirds of the patients had cystic component. Neither the clinical nor the radiographic presentation was a statistically significant predictor of the appropriate timing of intervention at time of tumor recurrence or progression. We found that the increase on the maximal dimension of the tumor compared to that at time of initial diagnosis was a statistically and clinically significant predictor of the appropriate timing of intervention. We also measured the increase at four different time points radiographic detection, overall intervention, immediate intervention and late intervention. Based on the ROC curves we have determined the cut-off points of the increase in the maximal dimension of the tumor at which we can confidently decide whether to intervene or not. So if a patient presents with asymptomatic radiographic recurrence, measure the progression of the tumor maximal dimension and if it's more than 30% intervene immediately. If it's less than 25% follow-up. If it's more than 25% and the less than 30% do MRI after three months and if it's a stable continue follow-up. If it's more than 5% progression then intervene immediately. In conclusion, the optimal management of recurrent progressive craniopharyngioma remains controversial. The vast majority of patients were asymptomatic, fine calcifications and subtotal resection alone without any adjuvant radiotherapy where the independent risk factors of tumor recurrence or progression. The increase in the maximal dimension of the tumor relative to that at time of initial diagnosis was found to be a statistically and clinically significant predictor of the appropriate timing of intervention. The aim of this novel algorithm is to determine the appropriate timing of intervention and to avoid any unnecessary tumor directed therapy. This paper has been accepted for publication in the Journal of Neurosurgery Peds and hopefully it will be published soon. Finally I'd like to thank Maddy, Steve, Dr. Michael Scott, Dr. Karen Marks and Dr. Lisa Bird for her mentorship of this work. Thank you.

Mohammad Vouda

postdoc fellow

Johns Hopkins University

pediatric craniopharyngioma

recurrence or progression