AUTHOR AND EDITOR INFORMATION

Section 1 of 12  

• Authors and Editors
• Introduction
• Differentials
• CT SCAN
• MRI
• Ultrasound
• Nuclear Medicine
• Angiography
• Intervention
• Further Reading
• Multimedia
• References

INTRODUCTION

Section 2 of 12  

• Authors and Editors
• Introduction
• Differentials
• CT SCAN
• MRI
• Ultrasound
• Nuclear Medicine
• Angiography
• Intervention
• Further Reading
• Multimedia
• References

Background

Hemangioblastomas are considered to be benign neoplasms and represent 1-2% of all primary central tumors. Historically, hemangioblastomas are linked to von Hippel-Lindau (VHL) disease.¹ In 1927, Arvid Lindau reported the connection between retinal angiomas and hemangiomas of the cerebellum.

VHL is an autosomal dominant condition involving chromosome 3 characterized by specific benign and malignant tumors with variable expressivity.^{2, 3} Cerebellar hemangioblastoma is the most common initial manifestation, affecting 64% of patients with VHL.⁴ In many series, cerebellar hemangioblastoma is the most important cause of mortality, affecting 47.7% of patients with VHL,⁵ followed by renal cell carcinoma.^{5, 6}

In patients with a positive family history, a single cerebellar hemangioblastoma is sufficient to make the diagnosis. If no known family history exists, at least 2 cerebellar hemangioblastomas or 1 hemangioblastoma plus 1 visceral tumor are necessary to justify the diagnosis of VHL.

Various organs can be involved (see Image 1).⁵ Of patients presenting with hemangioblastomas, 70% do not have a family history, and 3-25% of these patients have tumors associated with VHL. Hemangioblastoma appears to be associated more with VHL than previously reported, and it has been suggested that all patients with sporadic nonhereditary tumors should be evaluated for evidence of VHL disease.^{4, 6, 7}

For excellent patient education resources, visit eMedicine's Cancer and Tumors Center. Also, see eMedicine's patient education article Brain Cancer.

Related eMedicine topics:
von Hippel-Lindau Disease (Ophthalmology)
von Hippel-Lindau Disease (Pediatrics)
Von Hippel-Lindau Syndrome (Radiology)
Hemangioblastoma (Neurosurgery)
Hemangioblastoma, Spine

Related Medscape topics:
CME/CE  Targeted Therapies in Advanced Renal Cell Carcinoma (Slides With Transcript)
CME  Novel Targeted Therapies in Renal Cancer
Resource Center End-Stage Renal Disease
Resource Center Renal Cell Carcinoma
Specialty Site Radiology
Radiology CME and News

Pathophysiology

Hemangioblastomas are highly vascular tumors with 2 principal components: capillaries and stromal cells. The origin of the stromal cells is not well defined. The expression of transthyretin and transferrin has been reported in these cells, and the possibility exists that they may originate from embryonic plexus epithelium.⁸ The controversial origin has resulted in the classification of hemangioblastoma as a single entity in the category of tumors of uncertain histogenesis in the current World Health Organization system.⁹

Etiopathogenic studies on von Hippel-Lindau (VHL) demonstrate that the VHL gene (located on bands 3p25-26) acts as a tumor suppressor that binds the transcription factors elongin B and elongin C and inhibits transcriptional elongation. Germ-line mutation in patients with VHL and somatic mutations in different tumors have been identified.¹⁰ The development of tumors requires the inactivation of the 2 copies of the VHL gene. In patients with VHL, only 1 somatic mutation on the remaining allele is required for tumors to develop. In patients with sporadic cases, the 2 VHL gene mutations are seen. The hallmark of VHL tumors is high vascularization, which arises from increased levels of vascular endothelial growth factor (VEGF).

Patients with hemangioblastomas present with high levels of VEGF. The principal function of the VHL protein (pVHL) is negative regulation of hypoxia-inducible messenger RNA (mRNA), such as the mRNA encoding VEGF. The activity of pVHL has been linked to the targeting of specific proteins for proteolysis. When the VHL gene is mutated, absence of hypoxia-inducible transcription factor degradation (target of the proteolytic enzymes) is responsible for the accumulation of VEGF(see Image 2).¹¹

Hemangioblastomas are slow-growing tumors of endothelial cells with high tendency to be cystic (60%). The solid component is composed of capillaries, which are tightly packed blood vessels lined by endothelial cells. The blood vessels vary in size from capillary to cavernous. The absence of astrocytic endfeet and tight junctions can be found in the microvessels, which may lead to the breakdown of the blood-brain barrier, and have a role in cyst formation.¹²  Abundant intercapillary tissue is seen with swollen fat-laden endothelial cells that allow differentiation from angioma (see Images 3-4). A small cyst may exist in the mass, but more often, the cyst is situated adjacent to the tumor.¹³

The tumor does not have a capsule, and no mitotic changes are seen. The wall of the cystic component is composed of neural glial cells, with proteinaceous content that is rarely hemorrhagic. The cystic fluid is xanthochromic, with a concentration of amino acids, alkaline phosphates, and mucoproteins similar to blood, suggesting that they originate by diffusion from the vascular component of the solid tumor.¹⁴

Frequency

United States

Hemangioblastomas present most commonly in the posterior fossa, representing 7-12% of posterior fossa tumors in adults (including primary tumors and metastases). Almost 95% of infratentorial tumors are located in the cerebellum, primarily in the hemispheres, less frequently in the vermis, and even less commonly in the medulla oblongata and the spinal cord. Supratentorial hemangioblastomas are rare, with 85 reported cases, mostly in the cerebral hemispheres.

Hemangioblastomas have been described as intraventricular (in the third ventricle), from where cerebrospinal fluid seeding has been suggested to occur. Other less common locations include the pituitary gland, the corpus callosum, the basal ganglia, and the meninges.^{7, 15, 16}

Multiple hemangioblastomas occur in less than 5% of patients with sporadic cases and in as many as 20-25% of patients with von Hippel-Lindau (VHL). Hemangioblastomas usually are solitary. Multiple lesions are seen in 5% of patients without VHL disease and in 30-60% of patients with VHL disease.

• The rate of development of lesions is 1 lesion per 2.1 years.⁴

Mortality/Morbidity

Morbidity and mortality rates have been reduced as a result of diagnostic and therapeutic advances. Morbidity and mortality are related to the risk of rapidly enlarging cysts and the tendency for multiple recurrences. Morbidity and mortality rates are higher in patients with von Hippel-Lindau (VHL), in whom it is the first cause of death in many series, with an average rate of 40-60%.

• VHL penetrance is estimated to be 80-90% at age 65 years.²
• Enlargement of the cystic component can accompany pregnancy, leading to dramatic complications. Progesterone receptors have been demonstrated in the tumor, which can explain this phenomenon. Oral contraception is not recommended, but scientific proof does not exist to support the recommendation.
• The postoperative mortality rate is 7-10%, and it is higher in brainstem locations. Overall prognosis is good because 85% of patients survive 5-20 years after surgical removal of the tumor.^{4, 14}

Race

No racial predisposition has been reported for brain hemangioblastoma.

Sex

Hemangioblastomas are slightly more common in men than in women, with a male-to-female ratio of 1.3-2.6:1 in sporadic cases. Patients with hemangioblastomas related to von Hippel-Lindau (VHL) present with no sex differences in frequency.

Age

Hemangioblastomas occur more often in middle-aged adults aged 30-50 years. Patients with von Hippel-Lindau (VHL) present at a younger age than patients with sporadic cases, with an age range of 25-40 years.⁴

Anatomy

The nidus of the tumor abuts the pia matter, from which the tumor receives its vascular supply. The tumor is more frequently superficial than deep. Of hemangioblastomas, 60-70% are cystic, and the remainder are solid. Solid tumors are more common in the brainstem and supratentorial locations than elsewhere. The mural nodule is hypervascular and relatively small, less than 15 mm in diameter.

From a macroscopic point of view, hemangioblastomas can appear as solid or cystic tumors with mixed forms. Four types can be distinguished, as follows:

• Type 1, or the simple cyst form, is rare (6%) and characterized by a cyst with clear fluid and smooth walls and without evidence of a mural nodule at angiography or surgery.
• Type 2, or the macrocystic form, is the most frequent (65%) and characterized by a cyst of variable size with a mural nodule of approximately 1 cm.
• Type 3, or the solid form (25%), has a solid consistency with blurred limits and marked vascularization.
• Type 4, or the microcystic form (4%), is solid but contains small cysts of a few millimeters in size; therefore, 75% of infratentorial hemangioblastomas have a cystic component of variable size (see Image 5).

Clinical Details

Clinical symptoms depend on the site and size of the lesion because it is a slow-growing tumor. The patient can present with minor neurologic symptoms for months, followed by a sudden exacerbation. In infratentorial localizations, headache is the most common symptom as a result of increased intracranial pressure. Other symptoms include vomiting, giddiness, ataxia, and gait disturbances. Neurologic examination results are normal or include cerebellar signs, but hydrocephalus results in rapid decompensation with papilledema.

Polycythemia occurs in approximately 20% of cerebellar hemangioblastomas and is more common with solid hemangioblastomas.^{3, 5} Polycythemia is caused by tumoral production of erythropoietin. Subarachnoid or intra-axial hemorrhages are rare. Subarachnoid hemorrhages occur as a result of the subpial localization. Hemangioblastoma is the most common presenting manifestation of von Hippel-Lindau (VHL), revealing the disease in 30-50% of cases.³

Preferred Examination

Contrast-enhanced MRI is considered the best method for screening patients with von Hippel-Lindau (VHL) and the first evaluation used in symptomatic patients. However, preoperative angiography remains important for defining feeding vessels and aiding in embolization.^{17, 18}

Prior to MRI, contrast-enhanced CT scanning was performed frequently; however, beam-hardening artifacts produced by the petrous and vertebral bone limited its use.

MRI is superior to CT in the detection of vascular components of the tumor. Contrast-enhanced CT has the same sensitivity as nonenhanced MRI; however, it is inferior to contrast-enhanced MRI.¹⁷ Contrast-enhanced MRI permits the identification of small tumor nodules. In addition, MRI is helpful in separating cystic and solid components of the tumor from edema. Patients with VHL should be screened, and follow-up studies should be performed at 6 months.

Further follow-up studies should be performed at 1-year intervals to detect the development of additional tumors and monitor progression of existing lesions.¹⁷ Early treatment improves the outcome. Using MRI and CT at 1- to 2-year intervals, Conway et al identified 74% of lesions that required surgery before the patients became symptomatic.⁴

Limitations of Techniques

MRI is superior to nonenhanced CT in the detection of vascular components of the tumor.^{17, 19} Contrast-enhanced CT has the same sensitivity as nonenhanced MRI. The sensitivity of MRI increases with the use of gadolinium-based contrast material. Angiography is better in the detection of small (< 1 cm) vascular tumor components, and it is better for showing the vascular nature, supply, and drainage of tumors, compared with CT.²⁰ However, CT and MRI depict tumor cysts better.²¹

DIFFERENTIALS

Section 3 of 12  

• Authors and Editors
• Introduction
• Differentials
• CT SCAN
• MRI
• Ultrasound
• Nuclear Medicine
• Angiography
• Intervention
• Further Reading
• Multimedia
• References

CT SCAN

Section 4 of 12  

• Authors and Editors
• Introduction
• Differentials
• CT SCAN
• MRI
• Ultrasound
• Nuclear Medicine
• Angiography
• Intervention
• Further Reading
• Multimedia
• References

Findings

On CT scans, the tumor appears well circumscribed, solid, or cystic, with a mural nodule. Usually, the nodule is smaller that the cyst. This feature helps in differentiating it from cystic astrocytoma, which tends to have a larger nodule.

• In precontrast studies, the nodule is isoattenuating relative to the surrounding brain tissue, but it is hyperattenuating in occasional cases.
• After the administration of contrast material, the attenuation is equivalent or higher than that of the straight sinus, and the nodule enhances homogeneously.
• Commonly, the nodule abuts a pial surface. Multiplanar CT and MRI help in identifying the subpial localization (see Image 12).
• Generally, the cyst wall does not enhance, but occasionally, it can appear as a paper-thin line of enhancement that represents a combination of compressed cerebellar tissue and gliosis.
• Occasionally, the edge of the tentorium can be misinterpreted as an enhancing rim, with differentiation made by using coronal sections.
• The cyst is hypoattenuating on CT scans.²²

Degree of Confidence

CT shows all the clinically significant features of hemangioblastoma, along with secondary features such as hydrocephalus and edema.

False Positives/Negatives

False-negative results are found in patients with small nodules (<5 mm) that can be obscured by posterior fossa artifacts; therefore, CT is not a sensitive screening procedure.

False-positive results are rare. Metastases and cystic astrocytoma can appear similar. A diagnostic pitfall is the hemangioblastoma with a small central lucency, which can be interpreted as a necrotic metastasis. In this case, the ringlike enhancement of the necrotic nodule is thick and irregular.²²

MRI

Section 5 of 12  

• Authors and Editors
• Introduction
• Differentials
• CT SCAN
• MRI
• Ultrasound
• Nuclear Medicine
• Angiography
• Intervention
• Further Reading
• Multimedia
• References

Findings

Intracranial hemangioblastomas can manifest as 3 morphologic patterns based on the macroscopic pathology. These are well correlated with the MRI findings and include (1) cyst with a small mural nodule, (2) solid mass with a central cystic component, and (3) solid tumor without a cystic component.^{14, 18}

• A cyst with a small mural nodule is the most common presentation.
  • Cystic fluid surrounding the nodule is hyperintense on T1-weighted images and hyperintense on T2-weighted images. Characteristics of the fluid vary slightly related to protein content.
  • The mural nodule is isointense on T1-weighted images and demonstrates high signal on T2-weighted images.
  • After the administration of gadolinium-based contrast medium, the nodule shows prominent enhancement and the cyst does not enhance (see Image 8).¹⁴
• Solid masses with a central cyst behave similarly; therefore, the different morphologic patterns have been postulated to be part of the natural history of a solid tumor that develops a cystic component around or within it (see Images 6-7).^{23, 24}
• Solid hemangioblastomas occur less frequently than the other patterns (see Image 13).
• • The lesion is isointense or hypointense on T1-weighted images and hyperintense on T2-weighted images.
  • Transition from a solid tumor to a classic cystic tumor with a small mural nodule has been reported.
  • Occasionally, signal in solid tumor components can be heterogeneous on T1-weighted images, with areas of increased signal within the solid portion. These regions may represent lipid in the stromal cells or methemoglobin from hemorrhage.¹⁹
  • Gadolinium-based contrast agents (gadopentetate dimeglumine [Magnevist], gadobenate dimeglumine [MultiHance], gadodiamide [Omniscan], gadoversetamide [OptiMARK], gadoteridol [ProHance]) have been linked to the development of nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD). For more information, see the eMedicine topic Nephrogenic Fibrosing Dermopathy.

                  NSF/NFD has occurred in patients with moderate to end-stage renal disease
                  after being given a gadolinium-based contrast agent to enhance MRI or MRA
                  scans. NSF/NFD is a debilitating and sometimes fatal disease. Characteristics
                  include red or dark patches on the skin; burning, itching, swelling, hardening, and
                  tightening of the skin; yellow spots on the whites of the eyes; joint stiffness with
                  trouble moving or straightening the arms, hands, legs, or feet; pain deep in the
                  hip bones or ribs; and muscle weakness. For more information, see the
                  FDA Public Health Advisory or Medscape.

Degree of Confidence

MRI is the preferred screening study. Gadolinium enhancement increases the sensitivity.^{17, 21, 26, 27} MRI with gadolinium enhancement is the best study for screening, with the highest sensitivity and specificity compared with CT and nonenhanced MRI. Large studies are necessary to achieve a high degree of confidence; however, the advantages are obvious.

False Positives/Negatives

False-negative results can be seen with small lesions (<5 mm) and with delayed imaging because of the early enhancement of the lesions.

False-positive findings are rare. Most false-positive findings lead to an inadequate diagnosis. For solid and multiple enhancing lesions, metastases are the most important differential diagnosis; supratentorial lesions are more common in metastases. The visualization of associated abnormal vessels helps in differentiating hemangioblastomas from other cystic lesions.

ULTRASOUND

Section 6 of 12  

• Authors and Editors
• Introduction
• Differentials
• CT SCAN
• MRI
• Ultrasound
• Nuclear Medicine
• Angiography
• Intervention
• Further Reading
• Multimedia
• References

Findings

Gray-scale ultrasonography has been useful in the intraoperative evaluation of cerebellar and spinal hemangioblastomas by shortening and simplifying the surgical procedure. Most hemangioblastomas appear hyperechoic to adjacent neural tissue. Most studied cases have involved spinal hemangioblastomas. The use of ultrasonography is limited in brain lesions.

Doppler color flow images can be helpful in patients with isoechoic lesions, showing increase of vascularity as a tightly packed tangle of vessels. The technique can also demonstrate areas without flow that correspond to cystic areas.^{28, 29}

NUCLEAR MEDICINE

Section 7 of 12  

• Authors and Editors
• Introduction
• Differentials
• CT SCAN
• MRI
• Ultrasound
• Nuclear Medicine
• Angiography
• Intervention
• Further Reading
• Multimedia
• References

Findings

No helpful findings have been described for brain hemangioblastoma.

ANGIOGRAPHY

Section 8 of 12  

• Authors and Editors
• Introduction
• Differentials
• CT SCAN
• MRI
• Ultrasound
• Nuclear Medicine
• Angiography
• Intervention
• Further Reading
• Multimedia
• References

Findings

For many years, angiography was the primary diagnostic modality and still is a preoperative requisite in many patients. Infratentorial hemangioblastomas are evaluated by using vertebral angiography, which provides a selective opacification of both vertebral arteries by arterial femoral catheterization. Subtraction and magnification are necessary.²⁰

Angiography provides detection, exact localization, and definition of the vascularity of tumors in the vertebrobasilar system. Findings are specific, and the diagnosis can be made with confidence. Nodules usually stain intensely with either a homogeneous or mottled appearance (see Images 9-10). The tumor has irregular vessels. The largest tumors are associated with 1 or more enlarged feeding arteries and draining veins and can demonstrate arteriovenous shunting. When a cyst is present, it can be seen as an avascular area producing vascular displacement.^{20, 23}

Angiography is superior to CT in defining the vascular nodule and in characterizing occult and multiple lesions. The relationship of the nodule with the cyst is not demonstrated as well on angiograms as on MRI or CT scans.²⁰

Imaging of the neck with injection of each vertebral artery should be routine in patients with VHL complex or a posterior fossa hemangioblastoma. The blood supply is usually received through the pia mater; however, external carotid angiography provides valuable preoperative information in selected patients in whom a hemangioblastoma is superficial and abuts the dura (which is usually supplied by meningeal branches of the carotid system).

INTERVENTION

Section 9 of 12  

• Authors and Editors
• Introduction
• Differentials
• CT SCAN
• MRI
• Ultrasound
• Nuclear Medicine
• Angiography
• Intervention
• Further Reading
• Multimedia
• References

Surgical excision has been the treatment of choice for these tumors. Several factors should be considered in surgical planning, including the site, vascularity, recurrence, and multiplicity of the tumor. Usually, total resection of solid tumors is more difficult, making them more likely to recur, compared with cystic forms.³⁰

The surgeon must perform careful research on the mural nodule. If the cyst wall does not contain macroscopic tumor, it does not have to be removed. When the tumor is invading the brainstem, total excision is impossible. Postoperative mortality rates can be as high as 24%.³⁰

Hemangioblastomas are highly vascular tumors. When they are large, resection can be difficult because of excessive bleeding. The addition of a preoperative embolization procedure decreases the blood loss and allows more complete resection.³¹

Recurrence is the rule after partial removal, and the multicentric nature of the tumor also predisposes patients to recurrence, with an overall recurrence rate of 8-16%.

In cases of multiplicity, stereotactic radiosurgery was introduced as treatment in view of the otherwise high surgical risks.³² Stereotactic radiosurgery has been performed with satisfactory results. Small hemangioblastomas are ideal targets for radiosurgery.³³ Jawahar et al showed a 5-year rate of freedom from progression of 75% and a 10-year rate of 67%.³⁴

Fractionated radiation therapy has not been rigidly evaluated, but it appears to be partly effective. Sung et al reported good responses with 4500-5000 cGy.³¹

In patients with von Hippel-Lindau (VHL) disease, metastases from renal clear cell carcinoma can occur within a hemangioblastoma.^{20, 35, 36, 37, 38} Hematogenous metastases are more likely to occur in highly vascularized tissues, making the diagnosis difficult.³⁹ This situation is rare, and only a few cases have been reported. It is more common in meningiomas.⁴⁰

Medical/Legal Pitfalls

• Use of a gadolinium-based contrast agent is mandatory in the evaluation of hemangioblastomas because it increases the sensitivity for small, solid lesions.^{20, 26}
• When the diagnosis of hemangioblastoma is established, a careful evaluation for other small enhancing lesions should be performed because of the multiple lesions seen in some patients. The presence of multiple lesions has an important impact on the prognosis.⁶
• In hemangioblastomas located near the pia, differentiation from meningiomas can be difficult in certain patients. It is always important to look for characteristics that can help in diagnosing a vascular tumor, which is particularly important before surgery.
• In some patients, complete removal of the mural nodule is enough; however, in patients in whom the transition from solid tumor to cystic tumor with a mural nodule has been observed, the radiologist should perform careful follow-up imaging to assess the behavior of the lesions.

FURTHER READING

Section 10 of 12  

• Authors and Editors
• Introduction
• Differentials
• CT SCAN
• MRI
• Ultrasound
• Nuclear Medicine
• Angiography
• Intervention
• Further Reading
• Multimedia
• References

Indeterminate renal masses.
American College of Radiology.  1996 (revised 2007).  7 pages.  NGC:005996
 
Genetic cancer risk assessment and counseling: recommendations of the National Society of Genetic Counselors.
National Society of Genetic Counselors.  2004 Apr.  32 pages.  NGC:003601

EFNS guidelines on diagnosis and treatment of brain metastases: report of an EFNS Task Force. European Federation of Neurological Societies - Medical Specialty Society.  2006 Jul.  8 pages.  NGC:005485
 
Management of brain metastases: role of radiotherapy alone or in combination with other treatment modalities.
Program in Evidence-based Care.  2004 Mar 30.  35 pages.  NGC:003529

MULTIMEDIA

Section 11 of 12  

• Authors and Editors
• Introduction
• Differentials
• CT SCAN
• MRI
• Ultrasound
• Nuclear Medicine
• Angiography
• Intervention
• Further Reading
• Multimedia
• References

Media file 1:  Major clinical manifestations of von Hippel-Lindau disease.
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Media file 2:  Flow diagram illustrates the role of the von Hippel-Lindau protein in angiogenesis (modified from Richard, 2000).
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Media file 3:  Photomicrograph shows the classic microscopic appearance of a cerebellar hemangioblastoma with numerous capillaries and polygonal stroma cells shows vacuoles of cytoplasm and hyperchromatic nucleus (hematoxylin-eosin stain, high-power magnification). Courtesy of Dr Paul Fiedler.
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Media file 4:  Photomicrograph of a hemangioblastoma shows numerous vascular capillary networks with endothelial linings and stromal cells (hematoxylin-eosin stain, low-power magnification). Courtesy of Dr Paul Fiedler.
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Media file 5:  Diagram illustrates the cystic forms.
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Media file 6:  Patient with von Hippel-Lindau disease. T1-weighted gadolinium-enhanced MRI shows an enhancing nodule. Coronal T2-weighted MRI shows a small high-signal-intensity cyst.
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Media file 7:  Patient with von Hippel-Lindau disease (same patient as in Image 6 at 1-y follow-up). Coronal T1-weighted gadolinium-enhanced MRI shows an enhancing nodule that has grown in size adjacent to a low-signal-intensity cyst. T2-weighted MRI shows a cystic area that has increased in size, with high-signal-intensity characteristics.
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Media file 8:  Coronal and axial T1-weighted gadolinium-enhanced MRIs show a large cyst with a peripheral intense enhancing mural nodule.
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Media file 9:  Left, T1-weighted gadolinium-enhanced MRI with an enhancing nodule subpial in the cistern magna. Right, Angiogram obtained with a selective injection of the right vertebral artery shows an intense tumor stain in the arterial phase, without arteriovenous shunting.
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Media file 10:  On the left, a nonenhanced CT scan demonstrates acute bleeding in the posterior fossa. On the right, a selective angiogram of the vertebral artery shows an intense tumor stain.
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Media file 11:  Sagittal T1-weighted gadolinium-enhanced MRI shows a homogeneous intense enhancing tumor in the cistern magna.
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Media file 12:  T1-weighted postgadolinium MRI shows 2 subpial, enhancing nodules in a patient with von Hippel-Lindau disease. One nodule is small and present in the cistern magna; the other is in the cervical spine.
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Media type:  MRI

Media file 13:  Sagittal (top left) and coronal (top right) T1-weighted gadolinium-enhanced MRI images in a patient with von Hippel-Lindau disease presenting with 2 infratentorial hemangioblastomas. The larger tumor shows with cystic central areas. Bottom left, T1-weighted MRI shows that both lesions have low signal intensity. Bottom right, Abdominal axial image of the same patient shows multiple cysts in the pancreas.
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Media file 14:  Supratentorial hemangioblastoma proved by histologic analysis. Carotid arteriogram demonstrates a vascular, dense, tumor filled from the anterior cerebral vessels and not involving the sagittal sinus.
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Media type:  MRI

REFERENCES

Section 12 of 12

• Authors and Editors
• Introduction
• Differentials
• CT SCAN
• MRI
• Ultrasound
• Nuclear Medicine
• Angiography
• Intervention
• Further Reading
• Multimedia
• References

1. Butman JA, Linehan WM, Lonser RR. Neurologic manifestations of von Hippel-Lindau disease. JAMA. Sep 17 2008;300(11):1334-42. [Medline].
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3. Richard S, Campello C, Taillandier L, et al. Haemangioblastoma of the central nervous system in von Hippel-Lindau disease. French VHL Study Group. J Intern Med. Jun 1998;243(6):547-53. [Medline].
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9. Lach B, Gregor A, Rippstein P, Omulecka A. Angiogenic histogenesis of stromal cells in hemangioblastoma: ultrastructural and immunohistochemical study. Ultrastruct Pathol. Sep-Oct 1999;23(5):299-310. [Medline].
10. Tampieri D, Leblanc R, TerBrugge K. Preoperative embolization of brain and spinal hemangioblastomas. Neurosurgery. Sep 1993;33(3):502-5; discussion 505. [Medline].
11. Maxwell PH, Wiesener MS, Chang GW, et al. The tumour suppressor protein VHL targets hypoxia-inducible factors for oxygen-dependent proteolysis. Nature. May 20 1999;399(6733):271-5. [Medline].
12. Chen Y, Tachibana O, Hasegawa M, Xu R, Hamada J, Yamashita J, et al. Absence of tight junctions between microvascular endothelial cells in human cerebellar hemangioblastomas. Neurosurgery. 2006/09;59/(3):660-70.
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14. Ho VB, Smirniotopoulos JG, Murphy FM, Rushing EJ. Radiologic-pathologic correlation: hemangioblastoma. AJNR Am J Neuroradiol. Sep-Oct 1992;13(5):1343-52. [Medline].
15. Isaka T, Horibe K, Nakatani S, et al. Hemangioblastoma of the third ventricle. Neurosurg Rev. Oct 1999;22(2-3):140-4. [Medline].
16. Kachhara R, Nair S, Radhakrishnan VV. Sellar-sphenoid sinus hemangioblastoma: case report. Surg Neurol. Nov 1998;50(5):461-3; discussion 463-4. [Medline].
17. Filling-Katz MR, Choyke PL, Patronas NJ, et al. Radiologic screening for von Hippel-Lindau disease: the role of Gd-DTPA enhanced MR imaging of the CNS. J Comput Assist Tomogr. Sep-Oct 1989;13(5):743-55. [Medline].
18. Lee SR, Sanches J, Mark AS, et al. Posterior fossa hemangioblastomas: MR imaging. Radiology. May 1989;171(2):463-8. [Medline].
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Article Last Updated: Oct 1, 2008