Kaposiform hemangioendothelioma of skull base with dura invasion in a pediatric patient: a case report

Kaposiform hemangioendothelioma is an extremely rare vascular tumor which shows aggressive local growth. We present a case of rapid growing vascular skull tumor with dura invasion in a pediatric patient with neurofibromatosis type 1. A 14-year-old male complained of headache and dizziness for 1 mont...

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Bibliographic Details
Published in:Child's nervous system 2023-11, Vol.39 (11), p.3289-3294
Main Authors: Jung, Seong-Chan, Jung, Tae-Young, Lee, Tae-Kyu, Kim, Yeong Jin, Baek, Hee Jo, Kim, Sung Soon
Format: Article
Language:English
Subjects:
Case Report
Medicine
Medicine & Public Health
Neurosciences
Neurosurgery
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Summary:Kaposiform hemangioendothelioma is an extremely rare vascular tumor which shows aggressive local growth. We present a case of rapid growing vascular skull tumor with dura invasion in a pediatric patient with neurofibromatosis type 1. A 14-year-old male complained of headache and dizziness for 1 month after minor head trauma. Brain magnetic resonance imaging (MRI) revealed a 5-cm-sized tumor in the left frontotemporal bone with internal hemorrhage and cystic changes. The gross total resection of tumor was done. At the 7-month follow-up, brain MRI revealed a recurrent skull tumor with intracranial dura mass. He underwent second surgery, and the pathologic diagnosis was suggestive of Kaposiform hemangioendothelioma. For this vascular proliferative tumor, mTOR inhibitor was treated for 6 months, and there was the recurred nodular-enhancing mass along the sphenoid ridge. After additional 2 months of medication, the following MRI revealed a decreased nodular-enhancing mass.
ISSN:0256-7040
1433-0350
DOI:10.1007/s00381-023-06025-9