CNS Neoplasm Flashcards
Where do you most often find pediatric neoplasm vs adult neoplasm?
pediatric - 3:1 infratenorial (almost all primary)
adult - 3:1 supratentorial (75% primary)
What are key clinical presentations of increased intracranial pressure?
vomiting ESPECIALLY with waking in the morning
blurred vision (check for papilledema)
headache
how to tumors spread in CNS?
primary and metastatic tumors may spread WITHIN the CNS via CSF but primary CNS tumors never spread OUTSIDE the CNS
what is a paraneoplastic syndrome?
most commonly caused by small cell carcinoma of lung… so a tumor in a different location creates effects in the CNS or PNS (ie autoantibodies formed from the tumor that cross react with nervous system)
What are the 3 most common pediatric neuro neoplasms?
pilocytic astrocytoma
medulloblastoma
ependymoma
Pilocytic astrocytoma
most often in cerebellum (causing ipsi limb ataxia and falling toward that side)
often presents in older childood…. survival usually more than 10 years
cystic lesion with mural nodule of tumor is often present – where the cyst wall is glial tissue and not neoplastic
Medulloblastoma
incompletely differentiated fetal cells make it up resulting in a poorly differentiated neoplasm
often found in cerebellum in younger children (sonic hedgehog pathway problem)
solid tumor with “seeding throughout subarachnoid space”
-often travel in closely packed sheets, mitotically very active… gloomy prognosis
ependymoma
Usually found in 4th ventricle
solid tumor that is arranged in patterns with true ependymal rosettes or perivascular pseudorosettes…
poor prognosis
4 most common causes of adult neuro neoplasm (gliomas)
1) astrocytoma
2) oligodendroglioma
3) ependymoma
4) metastasis
Infiltrating astrocytoma + glioblastomas (grading system)
accounts for 80% of adult primary brain tumors; most common in astrocytoma category
Grade II = diffuse astrocytoma - pleomorphic nuclei
grade III = anaplastic astrocytoma - plus mitotic activity
grade IV = glioblastoma (usually arise due to an amplification of epidermal growth factor receptor gene) - plus necrosis and/or vascular endothelial proliferation
oligodendroglioma
often found in cerebral hemispheres
calcification is common
central nuclei and halo of clear cytoplasm (“fried-egg appearance”)
onset between 30-50 with slow growing tumors
ependymoma
most often occur in the lateral ventricles
Primary brain lymphoma
arises from B lymphocytes in the CNS
located deep in the cerebral hemispheres
signs/symptoms = headaches, seizures
*perivascular cuffing of tumor cells and diffuse infiltration of surrounding neural tissue
Most common CNS TOOMAH in immunosuppressed
Germ cell tumors
origin = ectopic germ cells
usually in the midline, especially in pineal/suprasellar region (good prognosis actually)
Meningiomas
often found attached to dura… does NOT invade adjacent brain tissue
psammoma bodies
more often affects women
often due to loss of chromosome 22 (NF2 gene)
peripheral nerve sheath tumors (eg schwannoma)
origin = schwann cells in peripheral nerves
Most common site is the CN8 at the cerebello-pontine angle
Signs and symptoms = “acoustic neuroma” involving tinnitus, hearing loss, and vertigo.
Tumors of Sellar Region (pituitary)
- *often present with endocrine problems**
- also present with CN3 or optic chiasm visual field deficits (ie bitemporal hemianopsia)
Pituitary adenomas are the most common… usually benign and slow growing
Craniopharyngioma - seprasellar epithelial tumor
Metastatic Neoplasm characteristics/etiology
Incidence: about 50% of adult CNS tumors are metastatic..
Typically spread hematogenously from especially the breast and lung
typically well-demarcated but often cause widespread edema
prognosis = poor (usually only months)
Neurofibromatosis type 1 (NF1)
autoDOM - one of the most common genetic disorders
often causes neurofibromas (benign nerve sheath tumors of the PNS) AND gliomas of the optic nerve
Neurofibromatosis type 2 (NF2)
autoDOM
bilateral acoustic shwannomas and multiple meningiomas
Tuberous Sclerosis
autoDOM
develop hamartomas and benign neoplasms involving the brain
Von Hippel-Lindau Disease
autoDOM
hemangioblastomas occur = tumors developing in the CNS from a vascular origin (especially in cerebellum and retina)
cysts often form in other organs
higher chance of renal cell carcinoma and pheochromocytoma