title: Multimodality management of a giant skull base hemangioendothelioma of the sphenopetroclival region.
abstract: A 20-year-old man presented with proptosis, nasal obstruction, vision loss and cavernous sinus syndrome, ongoing for 6 years. Imaging and biopsy confirmed a middle skull base epithelioid hemangioendothelioma arising from the left sphenopetroclival region with infratemporal fossa and intracranial-intradural extension into the left temporal lobe. Preoperative embolization of the left internal maxillary artery followed by a combined neurosurgical (front-temporal orbito-zygomatic craniotomy) and otorhinolaryngology (maxillary swing) approach was performed for tumor debulking. Postoperative radiotherapy and maintenance interferon chemotherapy was given to achieve a favorable outcome at 6 months follow-up. We describe the pertinent clinical, genetic, radiological and histopathological features, along with the available therapeutic modalities for a primary giant skull base hemangioendothelioma.
authors.affiliation: Department of Neurosurgery and Gamma Knife, All India Institute of Medical Sciences, Room 712, Ansari Nagar, New Delhi 110029, India.
authors.forename: Amol
authors.initials: A
authors.lastname: Raheja
authors.affiliation: Department of Neurosurgery and Gamma Knife, All India Institute of Medical Sciences, Room 712, Ansari Nagar, New Delhi 110029, India. Electronic address: surineuro@gmail.com.
authors.forename: Ashish
authors.initials: A
authors.lastname: Suri
authors.affiliation: Department of Otorhinolaryngology, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Shuchita
authors.initials: S
authors.lastname: Singh
authors.affiliation: Department of Otorhinolaryngology, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Rajeev
authors.initials: R
authors.lastname: Kumar
authors.affiliation: Department of Otorhinolaryngology, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Rakesh
authors.initials: R
authors.lastname: Kumar
authors.affiliation: Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Aruna
authors.initials: A
authors.lastname: Nambirajan
authors.affiliation: Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Meher C
authors.initials: MC
authors.lastname: Sharma
mesh_terms: D059248:Chemoradiotherapy / Q000379:methods; D003131:Combined Modality Therapy; D006390:Hemangioendothelioma / Q000628:therapy*; D006801:Humans; D008297:Male; D019635:Neurosurgical Procedures / Q000379:methods; D019292:Skull Base Neoplasms / Q000628:therapy*; D055815:Young Adult
keywords: Epithelioid; Hemangioendothelioma; Multimodality management; Skull base