Intracranial infantile hemangioma - rare entity and common pitfalls: a comprehensive multidisciplinary approach from neurosurgery, neurooncology and neuropathology

Objective: To review the rare entity of pediatric intracranial hemangiomas and discuss surgical pitfalls and pathophysiology in regard to cerebral edema formation. Materials and methods: We describe an extremely rare case of intracranial infantile hemangioma in a neonate with massive cerebral edema, surgically resected urgently because of acute herniation. We review the literature of 46 other pediatric cases of intracranial hemangioma, including congenital capillary hemangiomas. We analyze the data on age, sex distribution, tumor location, growth pattern, edema formation, histopathology, treatment, and outcome. Results: Isolated intracranial infantile hemangiomas in the neonatal period are extremely rare. Some but not all hemangiomas can be treated pharmacologically. Extensive cerebral edema is a frequent finding and can become an enormous problem at surgical resection. The pathogenesis of the edema formation in hemangiomas is complex and is yet not well understood. Conclusion: Surgical resection of intracranial hemangiomas is associated with a high morbidity, and pharmacological treatment should, if possible, always be considered first, at least for preoperative optimization. The severity of cerebral edema varies among intracranial hemangiomas, which may be an indicator of different molecular properties of the individual lesions. This implies that further sub-classification of intracranial hemangiomas may be necessary.