2. Case presentation
⢠A young asian man about 22year came for MRI
⢠Clinical presentation was in favor of
Cerebellar dysfunction :
Ataxia/ incordination/ gait disturbance
Headache
3. Investigations
⢠His routine lab investigations Blood & Urine were with
in normal limit
⢠MRI findings in different sequences:
A large left cerebellar cystic mass with non
enhanced wall. Cyst is with a small mural
nodule vividly enhanced and with flow voids.
Cyst is crossing midline and compressing IV
ventricle so causing dilatation of 3rd and lateral
ventricle
Impression: left cerebellar cyst crossing midline
and causing hydrocephalus
23. D/D Cerebellar cyst
⢠Haemangioblastoma
⢠Astrocytoma
⢠Sub acute infarction
⢠Vascular lesion
⢠Adult Meduloblastoma â rare much more solid
⢠Metastasis âusually old with primary
24. Haemangioblastoma
Cushing and Bailey introduced the term
Haemangioblastoma in 1928
Clinical symptoms:
Headache-70%
Hydrocephalus /ICH- 50%
Cerebellar dysfunction- 50-60%
altered mental state-10%
Polycythemia due to erythropoietin production
occurs in 5-40%
SYMPTOMS DEPENDS ON ANATOMIC LOCALIZATION
25. Haemangioblastoma
⢠It accounts for 1% of all intracranial tumor, in
isolation in 80% but is linked with
Von Hippel Lindau syndrome
⢠Most common in cerebellum
⢠In adult between 30-65% earlier withVHL
⢠Male : Female :: 1.3- 2.6
26. Site Haemangioblastoma
⢠Intracranial â 87-97%
95%------ posterior fossa
85% ----- cerebellar hemisphere
10% -----cerebellar vermis
5% -------medulla
5% -------supratentorial
Rarely up to CP angle
⢠Spinal â 3-13%
27. Histo-pathology Haemangioblastoma
⢠Mural nodule with cyst wall not demonstrating
tumor involvement in most cases
⢠Fluid of cyst often xanthochromatic
⢠Micro-vascular tumor composed of thin walled
vessels with surrounding stroma of connective
tissue
28. Haemangioblastoma on CT
⢠Cyst with nonenhancing wall
⢠Vividly enhanced mural nodule often has
prominent serpentine flow voids
⢠Calcification is not a feature
⢠Relatively mild edema and mass effect
** Mistaken for a low density glioma or gliomatous cyst
unless the mural nodule is identified in post enhanced
scan
29. Haemangioblastoma on MRI
⢠T1- Fluid filled cyst
Hypo intense to isointense mural nodule
vividly enhancing
⢠T2- Fluid filled cyst like CSF
Hyper intense mural nodule , flow voids due
to enlarged vessels at the periphery to cyst
31. Von-Hippel Lindau disease
⢠It is autosomal dominant hereditary syndrome
first described in 1926 by
Arvid Vilhelm Lindau
⢠Patient may present with--
1. cerebellar dysfunction- ataxia and in
coordination with or without hydrocephalus
2. Long H/O minor neurological problem or
sudden exacerbation
32. VHL contdâŚ..
⢠VHL includes retinal angiomatosis, CNS
haemangioblastoma and various visceral tumors most
commonly involving the kidneys and adrenal gland
⢠This syndrome is classified as PHAKOMATOSIS
although it does not include any cutaneous
manifestation.
⢠Itâs dominating mode of transmission compels
performing alerts screening of family members of patient
diagnosed with VHL
33. Diagnostic work up VHL
⢠Family history
⢠Detailed funduscopy
⢠Haematocrit & RBC count
⢠MRI with contrast
⢠Arteriography with DSA
⢠Spinal Angiography if spinal lesion on MRI
⢠Urine for Metanephrine- if +ve then 24 hrs VMA
⢠Abdominal CT scan esp. for pancreas, renal and
suprarenal
34. Vascular lesion
⢠Arterio -venous malformation
⢠Cavernoma
Both with or without bleed, confirmed on
Angiography
36. Pilocytic astrocytoma on MRI
Iso-Hypointense solid component compared to
adjacent brain on T1 and significantly Hyper
intense solid component on T2
37. Ependymoma on MRI
⢠Typically heterogenous mass in all modalities
Area of necrosis, calcification, cystic changes
and hemorrhage frequently seen
38. Diagnosis of our case
⢠On the basis of MRI imaging and clinical
picture most suggestive diagnosis of our case is
Haemangioblastoma
⢠Patient has been referred to specialist
39. Brain Tumor: Systemic approach
For analysis of potential brain tumor
Questions that need to be answered
1. Age of the patient
2. Localization- intra versus extra axial
Which anatomical compartment
Mid line crossing
3. CT or MRI- calcification, fat, cystic
4. Contrast enhancement
5. Effect on surrounding structure- mass effect, edema
6. Solitary or multiple
7. Pseudotumour
40. References
⢠Haemangioblastoma-Central nervous system
Dr Bruno Di Muzio and Dr Frank Gaillard et al
radiopaedia.org/article/
⢠Haemangioblastoma: Medscape Reference
emedicine.medscape.com/article
⢠Haemangioblastoma:
wikipedia.org/wiki/haemangioblastoma
⢠Brain tumor: systemic approach Robin Smithnis
and Walter Montanera Radiology Assistant
41. References contdâŚ..
⢠Haemangioblastoma: Neuroradiology
neuroradiology.ws/haemangioblastoma.htm
⢠Cerebellar haemangioblastoma: An unusual
cause of syncope
eradiology.bidmc.harvard.edu/learning
lab/mohamed.pdf
⢠Tumors of uncertain histogenesis-
haemangioblastoma Text book of Radiology
and Imaging vol 2 David Sutton