Supratentorial hemangioblastoma: clinical features, prognosis, and predictive value of location for von Hippel-Lindau disease

Mills, Steven A.; Oh, Michael C.; Rutkowski, Martin J.; Sughrue, Michael E.; Barani, Igor J.; Parsa, Andrew T.

doi:10.1093/neuonc/nos133

Cited by 51 publications

(50 citation statements)

References 110 publications

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“…Likewise there was edema along the optic tracts bilaterally, more so on the left side. The preoperative █ INTRODUCTION H emangioblastomas (HBLs) are benign vascular tumors of the central nervous system accounting for approximately 2% of all intracranial neoplasms (3). They are often associated with von Hippel-Lindau (VHL) disease (1).…”

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confidence: 99%

Optic nerve hemangioblastomas – a review of visual outcomes

Turel

Kucharczyk²,

Gentili³

2016

Turkish Neurosurgery

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ABSTRACThalos around objects as well as headaches. His visual acuity was 20/60 in the left eye and 20/20 in the right eye and visual field testing revealed concentric narrowing. Fundoscopic examination revealed left primary optic atrophy. Endocrine examination was normal.The magnetic resonance imaging (MRI) ( Figure 1A-D) showed a 1.8 cm extra-axial, well-defined, slightly lobulated lesion adjacent to the left anterior clinoid process and planum sphenoidale, compressing the optic nerve and the chiasm. The anterior cerebral artery (A1) was abutting the posterior surface of the tumor but was not encased. Most of the tumor was hypointense on T1-weighted images, enhanced uniformly, and showed heterogeneous intensity on T2-weighted images. There was a small cystic component to the tumor postero-inferiorly. The tumor did not have an obvious dural attachment and a dural-tail could not be identified. It was clearly separate from the pituitary gland and pituitary stalk. There was compression of the left optic nerve with no clear plane of separation. Likewise there was edema along the optic tracts bilaterally, more so on the left side. The preoperative █ INTRODUCTION H emangioblastomas (HBLs) are benign vascular tumors of the central nervous system accounting for approximately 2% of all intracranial neoplasms (3). They are often associated with von Hippel-Lindau (VHL) disease (1). Only 22 cases of optic nerve hemangioblastoma (ONH) have been reported in the literature. They often mimic meningiomas or optic nerve gliomas and are frequently misdiagnosed preoperatively. There has been only one other case of a HBL of the optic nerve producing optic tract oedema (9). We describe a case of an ONH without VHL, mimicking a meningioma preoperatively with optic tract edema. We review the visual outcomes of these patients as they have significant clinical implications. Such a review has not been done before in the literature. █ CASE REPORTA 67-year-old man presented with a 1-year history of episodic blurring of vision in the left eye. He also complained of seeing Supratentorial hemangioblastomas (HBLs) are exceedingly uncommon. They account for less than 5% of all central nervous system HBLs. The commonest location is the cerebral cortex. Rarely, they can involve the visual pathway. Most of the reports have focused on the differential diagnosis, since depending on the location they can often resemble optic nerve gliomas or meningiomas of the optic nerve sheath. In this study, we describe a 67-year-old man with progressive diminution of vision in the left eye with an optic nerve hemangioblastoma. There was no history of von Hippel-Lindau disease. We discuss the diagnostic challenges, and review the visual presentation and visual outcomes in these patients. This report, we believe, may add further clarity in counseling patients with an optic nerve lesion and the factors in surgical decision-making.

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confidence: 99%

Optic nerve hemangioblastomas – a review of visual outcomes

Turel

Kucharczyk²,

Gentili³

2016

Turkish Neurosurgery

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“…Among patients with supratentorial HB, 60% were diagnosed with VHL disease, and this condition is more common in VHL disease than infratentorial HB (33%)22,23,31). A significant association was found between HB tumors located in the sellar/suprasellar region and a diagnosis of VHL disease19). HBs may present as either isolated or multiple lesions.…”

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confidence: 93%

Meningeal Supratentorial Hemangioblastoma in a Patient with Von Hippel-Lindau Disease Mimicking Angioblastic Menigioma

Kim

Park

2013

J Korean Neurosurg Soc

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Hemangioblastomas are sporadic tumors found in the cerebellum or spinal cord. Supratentorial hemangioblastomas are rare, and those with meningeal involvement are extremely rare and have been reported in only approximately 130 patients. Here, we report the case of a 51-year-old female patient with supratentorial meningeal hemangioblastoma detected 5 years after surgical resection of an infratentorial hemangioblastoma associated with von Hippel-Lindau disease. Patients with von Hippel-Lindau syndrome are at risk for developing multiple hemangioblastomas, with new tumor formation and growth and possible meningeal infiltration. Regular lifelong follow-up in at-risk patients is recommended and should include the differential diagnosis of dural-based tumors such as angioblastic meningioma and metastatic renal cell carcinoma.

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“…Sporadic cases account for about two thirds of hemangioblastomas overall 4,19 but only for 40% of supratentorial hemangioblastomas. This trend is more particularly seen in suprasellar hemangioblastomas, with only 12 sporadic cases (including two suspected cases) reported.…”

Section: Discussionmentioning

confidence: 99%

“…1,2 Hemangioblastoma tends to occur in the infratentorial fossa, with only 2.9% of cases located in the supratentorial regions. 3 This tumor has a strong association with von Hippel-Lindau disease (VHLD), especially if located in the supratentorial regions, 4 particularly in suprasellar hemangioblastomas. Only 12 previous cases of suprasellar hemangioblastoma were identified as VHLD-unrelated sporadic cases (including two suspected cases).…”

Section: Introductionmentioning

confidence: 99%

Suprasellar Hemangioblastoma Unrelated to von Hippel-Lindau Disease Successfully Treated through Extended Transsphenoidal Approach: Diagnostic Value of Von Hippel-Lindau Disease Gene-Derived Protein

Wang

Ogawa

Ito

et al. 2016

J Neurol Surg A Cent Eur Neurosurg

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Hemangioblastoma tends to occur in the infratentorial regions and rarely in the supratentorial regions. This tumor is strongly associated with von Hippel-Lindau disease (VHLD), especially in the supratentorial regions, with only 12 cases of suprasellar hemangioblastoma unrelated to VHLD. However, all these cases were diagnosed based on clinical screening and confirmation of family history. We report a case of suprasellar hemangioblastoma that was successfully removed through an extended transsphenoidal approach and diagnosed as a sporadic case unrelated to VHLD by immunohistochemical examination.A 67-year-old woman had mild diabetes insipidus and a visual field defect. Head magnetic resonance imaging revealed a suprasellar tumor compressing the optic chiasm upward. The tumor was totally removed without complication through an extended transsphenoidal approach. Postoperative histologic examination disclosed large vacuolated stromal cells and rich capillary networks, and the diagnosis was established as hemangioblastoma. Clinical screening and confirmation of family history revealed no specific results, and additional immunohistochemical staining showed diffuse cytoplasmic expression of anti-VHLD gene-derived protein (pVHL).We emphasize that pVHL immunohistochemical evaluation would be wise to adopt, especially for patients with a high risk of VHLD related to younger age and supratentorial lesions.

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Supratentorial hemangioblastoma: clinical features, prognosis, and predictive value of location for von Hippel-Lindau disease

Cited by 51 publications

References 110 publications

Optic nerve hemangioblastomas – a review of visual outcomes

Optic nerve hemangioblastomas – a review of visual outcomes

Meningeal Supratentorial Hemangioblastoma in a Patient with Von Hippel-Lindau Disease Mimicking Angioblastic Menigioma

Suprasellar Hemangioblastoma Unrelated to von Hippel-Lindau Disease Successfully Treated through Extended Transsphenoidal Approach: Diagnostic Value of Von Hippel-Lindau Disease Gene-Derived Protein

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