Primary CNSTumors of Adults

BY SHANNON LARATONDA, MHS

Epidemiology

Half of all brain and spinal cord tumors are metastatic

The most frequent primary CNS tumors are glioblastoma multiforme and meningiomas

Primary malignant CNS tumors account for 2-3% of all cancer deaths in the U.S.

Clinical manifestations

Headache – often worse at night or early morning

Seizures – tumors involving cerebral cortex Mental changes – deficits in memory,

concentration, reasoning Focal neurological symptoms are related

to involvement of specific brain regions Symptoms related to increased ICP are

due to the presence of a space-occupying mass w/in the cranial cavity, blockage of CSF flow, and peritumoral edema

Primary Versus Metastatic Tumors

Primary Poorly circumscribed Usually single Location varies according

to specific type

Metastatic Well circumscribed Often multiple Usually located at the

junction between gray and white matter

Primary Adult CNS Tumors

Glioblastoma multiforme (Grade IV astrocytoma)

Meningioma Hemangioblastoma Schwannoma Oligodendroglioma Pituitary Adenoma

Glioblastoma multiforme (Grade IV astrocytoma

The most common CNS primary malignancy in adults

Most common location is white matter in the centrum semiovale

Characteristic histopath. feature is an area of necrosis surrounded by rows of neoplastic cells (pseudopalisading necrosis)

Aggressive tumors – poor prognosis, 1 yr. median survival

Originate from astrocytes and immunoreact w/ GFAP

Has a tendency to cross the midline by involving the corpus callosum – “butterfly glioma”

Glioblastoma multiforme

http://images.radiopaedia.org/images/940/67dcac388ad1cc69f7252e9ab44516.jpg

Head CT and MRI w/ contrast are best initial tests for any form of IC mass lesion

Meningioma

Common, typically benign Originates from meningothelial

cells of the arachnoid Women more than men Attach to dura and push

underlying brain w/o invasion

Composed of spindle shaped cells w/ indistinct borders; arranged in whorls or fascicles

Psammoma bodies are frequent Rx: resection and/or radiosurgery

Meningioma

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Hemangioblastoma

Most often cerebellar Assoc. w/ Von Hippel-Lindau

syndrome when found w/ retinal angiomas

Can produce EPO causing secondary polycythemia

Closely arranged, thin walled capillaries w/ minimal interleaving parenchyma

Benign, generally non-invasive Rx: surgical excision

Hemangioblastoma

http://http://www.virtualmedstudent.com/images/hemangioblastoma_MRI.jpg

Schwannoma

Originates from schwann cells of cranial or spinal nerves

Most frequent location is on the 8th CN at the cerebellopontine angle

Classical presentation of tinnitus and loss of hearing

Good prognosis after surgical resection or treated w/ stereotactic radiosurgery

Bilateral acoustic schwannomas are pathognomonic of NF Type 2

The neoplastic cells are immunoreactive with S-100 protein

Schwannoma

http://www.aboutcancer.com/an_am_0209.JPG

Oligodendroglioma

Most often in frontal lobes white matter

30-50 year old patients Often manifests w/ seizures Relatively rare, slow growing, 5-

10 year survival

Tend to recur after surgery and eventually degenerate into high-grade gliomas over time

Neoplastic cells similar to oligodendroglia w/ pronounced perinuclear halo

Oligodendroglioma

http://www.learnneurosurgery.com/uploads/1/6/6/8/16689668/9107439_orig.png

Pituitary Adenoma

Most commonly prolactinoma Manifests w/ bitemporal

hemianopia due to pressure on optic chiasm

Hyper or hypo-pituitarism are sequelae

Monomorphic neoplastic cells

Pituitary Adenoma

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References

Le, T., Bhushan, V., & Sochat, M. (2014). First Aid for the USMLE Step 1: Adult primary brain tumors. McGraw-Hill Education New York, NY.

Sanchez, H. and Barone, J. (2011). Kaplan Medical USMLE Step 1 Pathology: CNS Pathology. Kaplan Publishing. New York, NY.

Fischer, C. (June 2015). Master the Boards USMLE Step 2 CK: Oncology. Kaplan Publishing. New York, NY.