Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2020 Jan;45(1):67-72.
doi: 10.30476/ijms.2019.81095.

A Large Solitary Hemangioblastoma of the Lateral Ventricles: A Case Report and Literature Review

Affiliations
Case Reports

A Large Solitary Hemangioblastoma of the Lateral Ventricles: A Case Report and Literature Review

Alireza Tabibkhooei et al. Iran J Med Sci. 2020 Jan.

Abstract

Hemangioblastoma (HB) in the supratentorial region of the brain is rare and only a few cases are reported on intraventricular HB. HB of the lateral ventricles is even rarer. We present a case of a 30-year-old man with generalized tonic clonic seizures. The brain computed tomography showed a 5.5 cm heterogeneous mass extending into both lateral ventricles with partial enhancement. Based on the size and imaging features, we present the fourth documented case of a large solitary intraventricular HB. Our approach to this unique case and some treatment complexities are also described. Considering the rarity of the case and the patient's imaging features, the present study provides a better understanding of HB and recommends HB to be considered in the differential diagnosis of masses in the lateral ventricles. In addition, some preventable pitfalls in the treatment of such complex cases are described.

Keywords: Hemangioblastoma; Hydrocephalus; Lateral ventricle; Magnetic resonance imaging; Seizure; Solitary.

PubMed Disclaimer

Conflict of interest statement

Conflict of Interest: None declared.

Figures

Figure1
Figure1
Preoperative and postoperative images of a 30-year-old man with intraventricular hemangioblastoma. A 5.5 cm heterogeneous mass located in midline position extending into both lateral ventricles (a). An irregular enhancement, greater in the central portion of the mass, on axial without (b) and axial with gadolinium injection (c) views. The postoperative brain computed tomography scan revealed a small remnant of the tumor in the left lateral ventricle (d). The right-sided fronto-parietal chronic epidural hematoma on the last magnetic resonance imaging (axial view) was drained via one burr hole (e).
Figure2
Figure2
H&E ×200). Immunohistochemistry study of samples revealed positive reactivity for neuron-specific enolase (c). CD34 highlighted vascular channels (d). Ki67 was positive in 2-3% of tumoral cells (e). Histopathologic appearances and the results of immunohistochemical staining matched with HB diagnosis.

References

    1. Ammerman JM, Lonser RR, Dambrosia J, Butman JA, Oldfield EH. Long-term natural history of hemangioblastomas in patients with von Hippel-Lindau disease: implications for treatment. J Neurosurg. 2006;105:248–55. doi: 10.3171/jns.2006.105.2.248. - DOI - PubMed
    1. Richard S, Graff J, Lindau J, Resche F. Von Hippel-Lindau disease. Lancet. 2004;363:1231–4. doi: 10.1016/S0140-6736(04)15957-6. - DOI - PubMed
    1. Conway JE, Chou D, Clatterbuck RE, Brem H, Long DM, Rigamonti D. Hemangioblastomas of the central nervous system in von Hippel-Lindau syndrome and sporadic disease. Neurosurgery. 2001;48:55–62. doi: 10.1097/00006123-200101000-00009. - DOI - PubMed
    1. Mills SA, Oh MC, Rutkowski MJ, Sughrue ME, Barani IJ, Parsa AT. Supratentorial hemangioblastoma: clinical features, prognosis, and predictive value of location for von Hippel-Lindau disease. Neuro Oncol. 2012;14:1097–104. doi: 10.1093/neuonc/nos133. [ PMC Free Article ] - DOI - PMC - PubMed
    1. Matsumoto K, Kannuki S. Hemangioblastoma and von Hippel-Lindau disease. Nihon Rinsho. 1995;53:2672–7. - PubMed

Publication types

LinkOut - more resources