Download - Cerebellar cyst a case on mri
Case presentation
• A young asian man about 22year came for MRI
• Clinical presentation was in favor of
Cerebellar dysfunction :
Ataxia/ incordination/ gait disturbance
Headache
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 ventricleImpression: left cerebellar cyst crossing midline
and causing hydrocephalus
D/D Cerebellar cyst
• Haemangioblastoma
• Astrocytoma
• Sub acute infarction
• Vascular lesion
• Adult Meduloblastoma – rare much more solid
• Metastasis –usually old with primary
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
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
Site Haemangioblastoma
• Intracranial – 87-97%
95%------ posterior fossa85% ----- cerebellar hemisphere10% -----cerebellar vermis5% -------medulla5% -------supratentorial
Rarely up to CP angle
• Spinal – 3-13%
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
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
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
Haemangioblastoma on angiography
• Enlarged feeding arteries often dilated draining veins are demonstrated with dense tumor blush centrally
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 hydrocephalus2. Long H/O minor neurological problem or
sudden exacerbation
VHL contd…..
• 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.
• It’s dominating mode of transmission compels performing alerts screening of family members of patient diagnosed with VHL
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
Vascular lesion
• Arterio -venous malformation
• Cavernoma
Both with or without bleed, confirmed on Angiography
Pilocytic astrocytoma on MRI
Iso-Hypointense solid component compared to adjacent brain on T1 and significantly Hyper intense solid component on T2
Ependymoma on MRI
• Typically heterogenous mass in all modalities
Area of necrosis, calcification, cystic changes and hemorrhage frequently seen
Diagnosis of our case
• On the basis of MRI imaging and clinicalpicture most suggestive diagnosis of our case isHaemangioblastoma
• Patient has been referred to specialist
Brain Tumor: Systemic approach
For analysis of potential brain tumor Questions that need to be answered
1. Age of the patient2. Localization- intra versus extra axial
Which anatomical compartmentMid line crossing
3. CT or MRI- calcification, fat, cystic4. Contrast enhancement5. Effect on surrounding structure- mass effect, edema6. Solitary or multiple7. Pseudotumour
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
References contd…..
• 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