Abstract
Angiosarcoma is a rare form of soft tissue sarcoma originating from endothelial tissue, accounting for < 1% of all sarcomas. Primary epithelioid angiosarcomas of the central nervous system (CNS) are even more elusive, with only four reports described in the literature. In this article, we describe the first case in pediatric population, with a brief literature review regarding this entity. A 13-year-old girl presented to emergency services with raised intracranial pressure. MRI demonstrated a heterogenous lesion in the temporal lobe. She underwent emergency craniotomy and subtotal excision of the tumor. Eventually the patient developed multiple infarcts and succumbed post operatively. Pre-operative diagnosis on radiology is difficult considering the rarity of this entity and heterogeneity in radiological appearance. One needs to have a high degree of suspicion to consider angiosarcoma as a radiological differential. Overall prognosis remains poor. Early adjuvant treatment may improve overall survival.
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Tejasvi Singh Randhawa and Mayur Gopichand Gharat wrote the manuscript draft. Debyajyoti Chatterjee and Chirag Ahuja prepared slides, radiology, figures. Ashish Aggarwal and Raghav Singla edited and finalised the manuscript. All authors reviewed and approved the manuscript.
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Randhawa, T.S., Aggarwal, A., Chatterjee, D. et al. Pediatric primary intracranial angiosarcoma with epithelioid differentiation: a surgeon’s dilemma. Childs Nerv Syst 40, 267–271 (2024). https://doi.org/10.1007/s00381-023-06114-9
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DOI: https://doi.org/10.1007/s00381-023-06114-9