Figure 1
Non-enhanced CT brain
Non-enhanced brain CT shows an intra-axial infratentorial cystic lesion in the right cerbellum with a solid mural nodule arising from the posterior wall.
Haemangioblastoma
SectionNeuroradiology
Case TypeClinical Cases
Authors
M. Noeman (1)
(1) Radiology Resident, Department of Diagnostic and Interventional Radiology, Westpfalz Klinikum Kaiserslautern.
Connected authors
50 years, male
Clinical History
A 50-year-old male patient came to the ER complaining of an occipital headache that had increased during the past 3 weeks.
Neurological examination revealed Visual analogue scale (VAS) score of 8/10. The patient had brain CT (un-enhanced and enhanced) followed by MRI.
Neurological examination revealed Visual analogue scale (VAS) score of 8/10. The patient had brain CT (un-enhanced and enhanced) followed by MRI.
Imaging Findings
CT findings:
* Non-enhanced Brain CT: reveals an intra-axial infratentorial space-occupying lesion in the right cerebellum measuring about 4.5x3 cm consisting of a large cystic component (density similar to CSF) with a mural isodense nodule (compared to the brain tissue) arising from the posterior wall. There is mass effect with compression of the 4th ventricle.
* Enhanced Brain CT: the nodule shows a uniform and intense enhancement while there is no enhancement of the cystic wall.
MRI findings:
* Non-enhanced T1w and T2 w: the nodule has similar density to the brain tissues while the cystic component is isointense (compared to CSF).
* Enhanced T1w: the nodule shows a homogenous enhancement.
* Non-enhanced Brain CT: reveals an intra-axial infratentorial space-occupying lesion in the right cerebellum measuring about 4.5x3 cm consisting of a large cystic component (density similar to CSF) with a mural isodense nodule (compared to the brain tissue) arising from the posterior wall. There is mass effect with compression of the 4th ventricle.
* Enhanced Brain CT: the nodule shows a uniform and intense enhancement while there is no enhancement of the cystic wall.
MRI findings:
* Non-enhanced T1w and T2 w: the nodule has similar density to the brain tissues while the cystic component is isointense (compared to CSF).
* Enhanced T1w: the nodule shows a homogenous enhancement.
Discussion
A. Background:
- Haemangioblastomas are grade 1 tumours of borderline or uncertain behaviour according to the 2007 WHO Working Group. These tumours were categorised as "other neoplasms related to the meninges. [1]
- In 1926, Lindau described a cystic tumour of the cerebellum associated with retinal angiomatosis and tumours of the kidney. Cushing and Bailey [14] named this CNS tumour "haemangioblastoma, " thus emphasizing its neoplastic nature.
- It represents 1.1%-2.4 % of all intracranial space-occupying lesions. [2]
- It is a histologically benign tumour that usually occurs in young or middle-aged adults as a cerebellar tumour [2].
- Haemangioblastomas may occur as isolated lesions (66%) or in association with von Hippel Lindau disease (33%), a complex, autosomally dominant, inherited syndrome characterized by tumours or tumour-like lesions in several organs, including retinal angiomatosis, adrenal pheochromocytoma, cysts, and carcinomas of renal and pancreatic origin [3].
- Extracerebellar localization is rare.
- Favoured extracerebellar sites include cerebral hemispheres [1, 4], meninges [5], ventricles [6], medulla oblongata [7] and spinal cord [8].
B- Clinical presentation:
- Haemangioblastomas can cause neurologic deficits by direct compression or tumour-associated haemorrhage.
* Symptoms depend upon tumour location, and may include cerebellar ataxia, oculomotor nerve dysfunction, motor weakness, or sensory deficits. [9]
* Acute haemorrhage can be catastrophic and may cause rapid obstructive hydrocephalus, cerebellar tonsillar herniation, or brainstem compression [10, 11]. In these situations, emergency neurosurgical intervention is indicated.
- Patients with von Hippel-Lindau disease may have symptoms due to tumours in other organs.
C- Imaging perspective:
- Haemangioblastomas may be purely cystic (5%), purely solid (26%), cyst with a mural nodule (60%) and solid tumour with internal cysts (9%). [12]
- The size is variable from tiny to several centimeters. [13]
- The best diagnostic clue is an adult with intra-axial posterior fossa mass with cyst and an enhancing mural nodule. [12, 13]
- The best imaging tool is contrast-enhanced MR which is more sensitive than CT especially in small lesions.
- Absence of enhancement of the wall is important to differentiate haemangioblastomas from other cystic tumours presenting with wall enhancement like metastasis or glioblastoma multiforme. [19]
D- Treatment:
- Surgical resection offers definitive therapy for sporadic, isolated haemangioblastomas, particularly those arising in the cerebellum. [15]
- As it is highly vascular, patients frequently need a preoperative angiogram to identify feeding arteries. [15, 16, 17]
- For large lesions, embolization of feeding arteries with polymer microspheres, ethanol, or polyvinyl alcohol particles is typically performed prior to surgery. [16, 17]
E- Prognosis:
- The 10 years survival rate is 85%. The recurrence rate is about 20-33%. [18]
- Haemangioblastomas are grade 1 tumours of borderline or uncertain behaviour according to the 2007 WHO Working Group. These tumours were categorised as "other neoplasms related to the meninges. [1]
- In 1926, Lindau described a cystic tumour of the cerebellum associated with retinal angiomatosis and tumours of the kidney. Cushing and Bailey [14] named this CNS tumour "haemangioblastoma, " thus emphasizing its neoplastic nature.
- It represents 1.1%-2.4 % of all intracranial space-occupying lesions. [2]
- It is a histologically benign tumour that usually occurs in young or middle-aged adults as a cerebellar tumour [2].
- Haemangioblastomas may occur as isolated lesions (66%) or in association with von Hippel Lindau disease (33%), a complex, autosomally dominant, inherited syndrome characterized by tumours or tumour-like lesions in several organs, including retinal angiomatosis, adrenal pheochromocytoma, cysts, and carcinomas of renal and pancreatic origin [3].
- Extracerebellar localization is rare.
- Favoured extracerebellar sites include cerebral hemispheres [1, 4], meninges [5], ventricles [6], medulla oblongata [7] and spinal cord [8].
B- Clinical presentation:
- Haemangioblastomas can cause neurologic deficits by direct compression or tumour-associated haemorrhage.
* Symptoms depend upon tumour location, and may include cerebellar ataxia, oculomotor nerve dysfunction, motor weakness, or sensory deficits. [9]
* Acute haemorrhage can be catastrophic and may cause rapid obstructive hydrocephalus, cerebellar tonsillar herniation, or brainstem compression [10, 11]. In these situations, emergency neurosurgical intervention is indicated.
- Patients with von Hippel-Lindau disease may have symptoms due to tumours in other organs.
C- Imaging perspective:
- Haemangioblastomas may be purely cystic (5%), purely solid (26%), cyst with a mural nodule (60%) and solid tumour with internal cysts (9%). [12]
- The size is variable from tiny to several centimeters. [13]
- The best diagnostic clue is an adult with intra-axial posterior fossa mass with cyst and an enhancing mural nodule. [12, 13]
- The best imaging tool is contrast-enhanced MR which is more sensitive than CT especially in small lesions.
- Absence of enhancement of the wall is important to differentiate haemangioblastomas from other cystic tumours presenting with wall enhancement like metastasis or glioblastoma multiforme. [19]
D- Treatment:
- Surgical resection offers definitive therapy for sporadic, isolated haemangioblastomas, particularly those arising in the cerebellum. [15]
- As it is highly vascular, patients frequently need a preoperative angiogram to identify feeding arteries. [15, 16, 17]
- For large lesions, embolization of feeding arteries with polymer microspheres, ethanol, or polyvinyl alcohol particles is typically performed prior to surgery. [16, 17]
E- Prognosis:
- The 10 years survival rate is 85%. The recurrence rate is about 20-33%. [18]
Differential Diagnosis List
Cerebellar haemangioblastoma
Pilocytic astrocytoma
Cystic tumours with wall enhancement like metastasis or glioblastoma multiforme
Final Diagnosis
Cerebellar haemangioblastoma
References
[1] Louis DN, Ohgak i H, Wiestler OD (2007) The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol 114(2):97-109 (PMID: 17618441)
[2] Tarantino R, Isidori A, Raco A, Missori P (2000) Supratentorial hemangioblastoma in a patient with breast cancer: a case report. J Neurosurg Sci 44:137–139 (PMID: 11126448)
[3] Conway JE, Chou D, Clatterbuck RE, Brem H, Long DM, Rigamonti D (2001) Hemangioblastomas of the central nervous system in von Hippel-Lindau syndrome and sporadic disease. Neurosurgery Jan;48(1):55-62 (PMID: 11152361)
[4] Miller MH, Tucker WS, Bilbao JM. (1986) Supratentorial hemangioblastoma associated with von Hippel-Lindau disease: case report and review of the literature. Can Assoc Radiol J 37:35–37. (PMID: 2939080)
[5] Lee KR, Kishore PRS, Wulfsberg E, Kepes JJ (1978) Supratentorial leptomeningeal hemangioblastoma. Neurology 28:727–730 (PMID: 566874)
[6] Isaka T, Horibe K, Nakatani S, Maruno M, Yoshimine T (1999) Hemangioblastoma of the third ventricle. Neurosurg Rev 140–144 (PMID: 10547017)
[7] Djindjian M. (1986) Successful removal of a brainstem hemangioblastoma. Surg Neurol 25:97–100 (PMID: 3484562)
[8] Neumann HPH, Eggert HR, Weigel K, Friedburg H, Wiestler OD, Schollmeyer P (1989) Hemangioblastomas of the central nervous system: a 10 year study with special reference to von Hippel-Lindau syndrome. J Neurosurg 70:24–30 (PMID: 2909683)
[9] Roonprapunt C, Silvera VM, Setton A, et al. (2001) Surgical management of isolated hemangioblastomas of the spinal cord. Neurosurgery 49:321 (PMID: 11504107)
[10] Wakai S, Inoh S, Ueda Y, Nagai M. (1984) Hemangioblastoma presenting with intraparenchymatous hemorrhage. J Neurosurg 61:956 (PMID: 6541682)
[11] Adegbite AB, Rozdilsky B, Varughese G. (1983) Supratentorial capillary hemangioblastoma presenting with fatal spontaneous intracerebral hemorrhage. Neurosurgery 12:327 (PMID: 6682491)
[12] Smirniotopoulos JG1, Murphy FM, Rushing EJ, Rees JH, Schroeder JW. (2007) From the Archives of the AFIP Patterns of Contrast Enhancement in the Brain and Meninges1. RadioGraphics Mar-Apr;27(2):525-51 (PMID: 17374867)
[13] Maiuri F. (1988) Cysts with mural tumor nodules in the cerebral hemispheres. Neurosurgery 22:703–706 (PMID: 3374781)
[14] Cushing H, Bailey P. (1928) Hemangiomas of Cerebellum and Retina (Lindau\'s Disease): With the Report of a Case. Trans Am Ophthalmol Soc 26:182-202. (PMID: 16692792)
[15] Eskridge JM, McAuliffe W, Harris B, et al. (1996) Preoperative endovascular embolization of craniospinal hemangioblastomas. AJNR Am J Neuroradiol 17:525 (PMID: 8881249)
[16] Tampieri D, Leblanc R, TerBrugge K. (1993) Preoperative embolization of brain and spinal hemangioblastomas. Neurosurgery 33:502 (PMID: 8413884)
[17] Lonser RR, Heiss JD, Oldfield EH. (1998) Tumor devascularization by intratumoral ethanol injection during surgery. J Neurosurg 88:923 (PMID: 9576266)
[18] Symon L, Murota T, Pell M, Bordi L. (1993) Surgical management of haemangioblastoma of the posterior fossa. Acta Neurochir (Wien) 120: 103-110 (PMID: 8460560)
[19] Sharma V, Prabhash K, Noronha V, Tandon N, Joshi A. (2013) A systematic approach to diagnosis of cystic brain lesions. South Asian J Cancer 2(2): 98–101. (PMID: 24455569)
Case information
| URL: | https://www.eurorad.org/case/9428 |
| DOI: | 10.1594/EURORAD/CASE.9428 |
| ISSN: | 1563-4086 |
Figure 2
Contrast-enhanced CT of the brain
Contrast enhanced brain CT - at the same level - showing enhancement of the nodule while no enhancement of the wall of the cyst is seen.
Figure 3
Axial non-enhanced T2-weighted MRI of the brain
In this axial T2w MRI the nodule is seen nearly isotense (compared to the brain tissues) while the cystic component is isointense to CSF. There is mass effect with compression of the 4th ventricle.
Figure 4
Enhanced T1-weighted coronal MRI
The nodule shows homogenous enhancement.
Non-enhanced CT brain
Non-enhanced brain CT shows an intra-axial infratentorial cystic lesion in the right cerbellum with a solid mural nodule arising from the posterior wall.
Contrast-enhanced CT of the brain
Contrast enhanced brain CT - at the same level - showing enhancement of the nodule while no enhancement of the wall of the cyst is seen.
Axial non-enhanced T2-weighted MRI of the brain
In this axial T2w MRI the nodule is seen nearly isotense (compared to the brain tissues) while the cystic component is isointense to CSF. There is mass effect with compression of the 4th ventricle.
Enhanced T1-weighted coronal MRI
The nodule shows homogenous enhancement.