18 year old girl with headaches, nausea and vomiting for one month. She has been seen multiple times in the ER (7 visits) with no diagnosis nor significant relief. A CT scan of brain obtained on last visit ...
No focal deficit.
Complex enhancing mass within the 4th ventricle. Mass contains cystic elements. The mass obstructs the 4th ventricle outflow resulting in non-communicating hydrocephalus.
â€¢ Choroid Plexus papilloma
â€¢ Pilocytic astrocytoma
Dx: Ependymal neoplasms, Ependymoma WHO grade II
Dx Confirmed by: Surgery and Pathological examination
Ventricular drain (shunt) placement followed by surgery.
Radiation and/or Chemotherapy pending.
Cell of Origin:
Ependymal cells lining the ventricle and central canal of the cord
Ependymoma - Gr 2
Anaplastic Ependymoma - Gr 3
Myxopapillary - Gr 1
Subependymoma - Gr 1
Neurofibromatosis Type 2
70% in fourth ventricle
70% under the age of 20
soft intracavitary mass
sharply demarcated, perivascular pseudorosettes, ependymal rosettes (canals),
soft grayish sharply marginated, rarely invade brain
in spinal cord - sharply demarcated central masses
heterogeneous intraventricular mass
small chunks of calcification
small 'cystic' areas
intraspinal are sharply demarcated, often with a hemosiderin 'cap'
Prognosis and Treatment:
50% or more at 5 years
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