intracranial mass
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Posterior Fossa Mass
Leo SmithDecember, 2004
Patient Presentation
26-year old male from small western Oregon town~2.5 week history of headache, more painful in the morning. No significant improvement w/ pain medication.“Throbbing” behind right ear~2 week history of imbalance, nausea, and emesis.Episodes of sudden deafness.Right arm numbness.Blurred vision and diploplia.CT in home town showed right cerebellar mass.Patient referred to OHSU.
Patient Presentation (cont’d)
Family history of multiple cancers, type unknown.
Physical examination: Alert, oriented x3 Normal pupils and EOM. Normal visual fields Slight decrease in sensation in R side of body No other contributory physical exam findings
Findings: MRI, T2 Weighted
Hyperintense mass lesion within the right cerebellar hemisphere, ~ 4 x 3 x 3 cm.
Mild downward displacement of cerebellar tonsils, especially on right
Some effacement of prepontine cistern
MRI, T2 Weighted Imaging
Findings: T1 Weighted MRI
Isointense mass w/ position and size as described above
MRI, T1 Weighted, w/o Contrast
Findings: MRI w/ Contrast
Approximately 1 cm enhancing nodule within mass
MRI, T1 w/ Gadolinium Contrast
Findings on Angiography
Hypervascular mass, 8 x 12 mm, within right cerebellum
Hypervascular mass is being supplied by branches from the right anterior inferior cerebellar artery, and the right posterior inferior cerebellar artery.
Angiographic Imaging
R Vertebral Artery
Basilar Artery
Localization of Vascular Mass
Location of hypervascular massCorresponds w/ location of enhancing nodule
Differential Diagnosis
Hemangioblastoma most common posterior fossa primary neoplasm in adults
Juvenile pilocytic astrocytoma Most common posterior fossa primary neoplasm in children
Metastatic tumor Most common intra-axial, infratentorial tumor in adults Typically have a significant amount of edema surrounding
tumor
Ganglioglioma Typically show calcification
Diagnosis
Hemangioblastoma, based on: Imaging characteristics and location of mass Age of patient
Cannot rule out juvenile pilocytic astrocytoma (JPA) at this time.
Hemangioblastoma and JPA can be distinguished on pathology exam
Metastasis unlikely because: Young age of patient Absence of signs of primary neoplasm elsewhere in body Limited extent of surrounding edema seen in this patient
Treatment
Evidence of neurological deficit or hemorrhage are indications for surgical resection
Pre-surgery embolization of feeding arteries, where possible
Should consider adjuvant radiotherapy in cases with positive surgical margins or incomplete resection
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Typical Surgical Finding
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Outcome for This Patient
Nodule and surrounding cyst successfully resected
Pathology confirmed diagnosis of hemangioblastoma
Relatively uneventful post-op stay
Discharge to home post-op day 5.
Hemangioblastomas
Diagnostic Characteristics of Hemangioblastomas (1)
A. MRI1. Enhancing nodule associated with cyst, located in the cerebellum.
2. Smaller HBs (<10 cm) can appear isointense on T1 and hyperintense on T2 imaging.
3. Can also present as uniformly enhancing lesion on surface of or within spinal cord.
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Diagnostic Characteristics of Hemangioblastomas (2)
B. Angiography: Highly vascularlized mass
C. Blood count: In 15-20% of pts. with hemangioblastoma there is a perineoplastic erythrocytosis (w/ elevated RBC and hematocrit). This is due to extra-renal erythropoietin production.
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Characteristics of Hemangioblastoma
Hemangioblastomas (HBs) account for 7-10% of all posterior fossa primary neoplasms
Most are found in young adults
Within the neuraxis, hemangioblastomas occur most often in the cerebellum, but also occur in Spinal cord Medulla Pituitary stalk
Important Considerations w/ Hemangioblastoma (1)
~25% of pts. w/ CNS hemangioblastomas have von Hippel-Lindau (vHL) disease
Sporadic HBs typically appear in 5th or 6th decade; vHL-associated HBs typically appear in 3rd or 4th decade. Therefore, if pt. under 50 is found to have HB, pt. should be screened for vHL
Important Considerations w/ Hemangioblastoma (2)
vHL is an autosomal dominant inheritable disease
vHL predisposes to multiple tumors, including: Neuraxial HBs Retinal HBs Renal cell cysts and renal cell carcinoma Pheochromocytomas Pancreatic cysts and pancreatic serous cystadenomas Pancreatic and neuroendocrine tumors
vHL can be diagnosed by genetic testing, or by meeting certain criteria
Is it von Hipple-Lindau?
Pt. likely has vHL if:A. Multiple CNS hemangioblastomas (presumptive
vHL),
OR
B. Neuraxial hemangioblastomas with other neoplasms outside CNS
Diagnostic Criteria for vHL1
A. More than one HB of the CNS or retina, or isolated HB in association with any of following:
pheochromocytoma renal carcinoma pancreatic involvement
B. A first-degree relative w/ vHL and any one manifestation
Follow-Up for Pts. w/ Established vHL1
1) Annual physical examination and urine test
2) Annual ophthalmology exam
3) MRI of brain q 3 years to 50 y.o., q 5 years thereafter
4) Annual abdominal MRI
5) Annual 24-hour urine test for vanillylmandelic acid (VMA)--screen for pheochromocytoma
Screening Recommendations for Relatives at Risk1
1) Annual physical and urine test after 5 y.o.
2) Annual ophthalmology exam from 5-60 y.o.
3) Brain MRI q 3 yrs. from 15-40 y.o., q 5 yrs. thereafter
4) Annual abdominal MRI from 20-60 y.o.
5) Annual 24-hour urine test for VMA
References
1. Maddock IR, et al. A genetic register for von Hippel-Lindau disease. J Med Genet 33(2):120-127, 1996.
2. Slater A, et al. The Natural History of Cerebellar Hemangioblastomas in von Hippel-Lindau Disease. Am J Neuroradiol, 24:1570-1574, Sep 2003.
3. Grainger RG. Grainger & Allison’s Diagnostic Radiology: A Textbook of Medical Imaging, 4th ed. p 2336 Churchill Livingstone, Inc. Edinburgh, UK, 2001.
4. Choyke PL, et al. Von Hippel Lindau Disease: Genetic, Clinical and Imaging Features. Radiology (March) 146:629-642, 1995.
5. Wong ET, et al. Hemangioblastoma. UpToDate. www.uptodate.com.
The End
Embolization of Hemangioblastoma
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Enhancing Nodule and Cyst
Gd enhancing noduleAssociated with cyst.
T1 weighted MRI with Gd contrast
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Hemangioblastoma, MRIT1 vs. T2
T1: Isointense T2: Hyperintense
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Hemangioblastoma Spinal Lesion
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Angiogram of vascularized mass
Mass with high vascularity
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