Preoperative embolization and surgical resection of a hypervascular pediatric hemangioendothelioma with intra -extracranial extension: a case report

Abstract Background Hemangioendothelioma is a rare vascular tumor with biological behavior intermediate between hemangioma and angiosarcoma. It occurs more frequently in young adults than in children and most commonly involves the liver, lungs, lymph nodes, and bones. Combined intra- and extracranial involvement is exceptionally rare. The rarity of such cases necessitates careful analysis of the available literature to guide diagnostic evaluation, treatment strategies, and prognostic assessment. Case description We present a clinical case of a 10-year-old girl with a giant hypervascular epithelioid hemangioendothelioma of the cranial vault. The tumor was first diagnosed in 2023 as an intracranial mass in the frontotemporal region associated with bone destruction. Subsequently, the lesion demonstrated progressive aggressive growth, reaching giant dimensions with extension into the fronto-orbital, parietal, and temporal regions. Magnetic resonance imaging (MRI) and computed tomography (CT) revealed a hypervascular tumor measuring 13.5 × 7.0 × 12.4 cm with combined intra- and extracranial components. Digital subtraction angiography (DSA) confirmed tumor vascularization from branches of the external carotid artery. The feeding vessels were embolized using a liquid embolic agent. On the following day, radical surgical resection of the tumor was performed. Histopathological examination confirmed the diagnosis of epithelioid hemangioendothelioma. The postoperative course was uneventful, with gradual regression of clinical symptoms and no neurological deficit. Conclusion This case highlights the effectiveness of preoperative embolization as an adjunct to surgical resection of hypervascular cranial tumors in pediatric patients, significantly reducing intraoperative blood loss and related complications. Keywords: epithelioid hemangioendothelioma; preoperative embolization; gross total resection; intra-extracranial extension .