Giant Cerebellar Cavernomas in Pediatric Patients: Systematic Review with Illustrative Case Mairre James S. Gaddi, MD, Juan Silvestre G. Pascual, MD, Eric Dennis C. Legaspi, MD, Peter P. Rivera, MD, and Abdelsimar T. Omar II, MD Introduction: Giant cavernomas (GCMs) are angiographically occult vascular mal- formations of the central nervous system measuring at least 4 cm in diameter. These are rare lesions, especially in the cerebellum. As such, the clinical and radiologic fea- tures, surgical management, and outcomes of treatment for this condition are unknown. Methods: We performed a systematic review of SCOPUS and PubMed databases for case reports and case series of histopathologically proven GCMs aris- ing from the cerebellum in the pediatric population, and included a case managed at our center. Data on clinical presentation, imaging features, surgical treatment and outcomes of management were collected. Results: A total of 10 cases were iden- tified from the systematic review, including our case report. The patients’ median age was 6 months, with a slight male sex predilection (1.5:1). The most common neurologic manifestations were bulging fontanelle in infants, and headache and focal cerebellar deficits in older children. Surgical excision was done in all cases, while 50% of the cases had CSF diversion surgery done prior to excision. In cases with reported outcome, complete neurologic recovery was achieved in seven while partial recovery was reported in one case. No deaths were reported at median fol- low-up of 12 months. Conclusion: Our case report and systematic review show that giant cerebellar cavernoma is a rare differential diagnosis for symptomatic hemor- rhagic cerebellar masses in the pediatric age group. Mainstay of treatment is surgi- cal excision, which can be associated with favorable outcome in most cases. Key Words: Cavernous malformation—Giant—Pediatric—Cerebellum— Cavernous angioma—Cavernoma © 2020 Elsevier Inc. All rights reserved. Introduction Cavernomas or cavernous malformations (CM) are angiographically occult vascular malformations in the central nervous system. They are composed of thin- walled, dilated vascular channels lined by a single layer of endothelium, separated by a collagenous stroma and lacking any intervening brain parenchyma. 1À3 They occur in about 0.37%À0.9% of the population based on autopsy and imaging studies. 2À9 In children, prevalence is estimated to be between 0.37% and 0.53%. 3,10,11 Caverno- mas vary in size, ranging from a few millimeters to a few centimeters. Giant cavernomas (GCMs) are a distinct sub- group of these lesions. The definition for a GCM varies, but most authors use a size cut-off of 4 cm. 3,12À15 GCMs are rare lesions, with a recent review reporting only 44 cases in the literature. Most are commonly located in the supratentorial compartment with majority arising in the frontoparietal lobe. 14 Even rarer are GCMs located in the cerebellum. As such, the clinical manifestations, optimal surgical and medical treatment and outcomes of treatment for this pathology remain unknown. We report a case of giant cerebellar cavernoma in a pedi- atric patient managed at our center. We also performed a systematic review of the literature for case reports and case series of cerebellar cavernous malformations in the pediat- ric population and discuss clinical presentation, imaging features, management options and outcomes. From the Division of Neurosurgery, Department of Neurosciences, University of the Philippines Manila À Philippine General Hospital, Taft Avenue, Manila, Philippines. Received July 13, 2020; revision received August 18, 2020; accepted August 20, 2020. Corresponding author. E-mail: atomar@up.edu.ph. 1052-3057/$ - see front matter © 2020 Elsevier Inc. All rights reserved. https://doi.org/10.1016/j.jstrokecerebrovasdis.2020.105264 Journal of Stroke and Cerebrovascular Diseases, Vol. 29, No. 11 (November), 2020: 105264 1