Neoplasm

Question 1

What is paraneoplastic cerebellar degeneration?

Answer 1

Presents with acute or subacute onset of pancerebellar syndrome with truncal, gait, and limb ataxia, dysarthria, and disturbances of of ocular motility. It evolves over several weeks, then stabilizes, but leaves the patient profoundly disabled. Typically associated with underlying gynecologic or small cell lung cancer. May have autoantibodies (e.g. anti-Yo and anti-Hu)

Question 2

What is the treatment for glioblastoma multiforme (aka grade IV astrocytoma)?

Answer 2

Surgical resection followed by radiation with concurrent temozolomide, followed by adjuvant temozolomide for 6 months. Nitrosoureas (“-mustine” suffix) can be added for combination therapy

Question 3

What cancer type causes a “butterfly” pattern on MRI?

Answer 3

Glioblastoma multiforme

Question 4

What is the genetic signature of oligodendrogliomas?

Answer 4

Codeletion of chromosomal arms 1p and 19q

Question 5

What is the pathology of ependymomas?

Answer 5

Perivascular pseudorosettes

Question 6

Where do ependymomas most commonly occur in children vs adults?

Answer 6

Children: in the CNS, commonly within the 4th ventricle - can lead to increased ICP
Adults: spinal canal - can cause conus medullaris or cauda equina syndromes

Question 7

What are characteristics of medulloblastomas?

Answer 7

Usually at the medullary velum of the 4th ventricle (can lead to hydrocephalus). More common in children. Neuroectodermal tumor.

Question 8

What are characteristics of meningiomas?

Answer 8

Almost always benign. Arise from the meningothelial (mesodermal) cells of the dura matter. Second most common PBT after GBM. Patients with neurofibromatosis 2 are at increased risk. Hystology shows sheets of plump, uniform meningothelial cells with the tendency to form whorls. Progesterone receptors are common. Treated with resection

Question 9

What type of lymphoma is CNS lymphoma typically?

Answer 9

Intermediate to high-grade non-Hodgkin lymphomas of B cell origin.
It is usually treated with chemotherapy plus radiation. Dementia occurs in more than 50% patients that survive more than 18 months post radiation therapy

Question 10

What CNS tumors are associated with NF2?

Answer 10

Bilateral vestibular schwannomas, meningiomas, and intramedullary ependymomas

Question 11

What type of brain metastases bleed easily?

Answer 11

Melanoma, renal cell carcinoma, and choriocarcinoma

Question 12

What type of cancers tend to metastasize to the brain?

Answer 12

Lung, melanoma, renal cell carcinoma, breast, and colon. Lung cancer accounts for 2/3 of cases

Question 13

What is paraneoplastic cerebellar degeneration and what is the underlying cause?

Answer 13

It is a pancerebellar syndrome the manifests as ataxia, dysarthria, and nystagmus with acute or subacute onset that has rapid progression over several weeks, then stabilization. Usually due to an underlying gynecologic or breast malignancy

Question 14

Why can increased ICP due to and expanding tumor cause bilateral CN VI palsies (problems with abduction bilaterally)?

Answer 14

The increased ICP can stretch the 6th nerves

Question 15

What type of brain mets are likely to cause hemorrhage (and thus have an especially bad prognosis)?

Answer 15

Malignant melanoma and choriocarcinoma

Question 16

Where is the most common site for medulloblastoma occurrence?

Answer 16

Cerebellum - leads to ataxia

Question 17

What is Parinaud syndrome?

Answer 17

Caused by lesions of the dorsal midbrain in the area of the superior colliculus - e.g. tumors in the pineal region (e.g. pineocytomas, germinomas, gliomas). Causes loss of vertical gaze, loss of pupillary light reflex, lid retraction, and convergence-retraction nystagmus (eyes appear to jerk back into the orbit on attempted upgaze)

Question 18

What is paraneoplastic cerebellar degeneration?

Answer 18

Characterized by progressive ataxia, dysarthria, and nystagmus. Due to an underlying cancer (small cell carcinoma of the lung, ovarian carcinoma, and lymphoma, in that order). Imaging may eventually show cerebellar atrophy due to loss of Purkinje cells (caused by anti-Yo antibodies directed against Purkinje cells)

Question 19

What CNS complications are seen in hypercalcemia (e.g. lung cancer releasing parathyroid-related peptide)?

Answer 19

Increased serum Ca+ causes decreased membrane excitability, leading to fatiguability, lethargy, generalized weakness, and areflexia. Can lead to coma and convulsions

Question 20

What is Foster-Kennedy syndrome?

Answer 20

A constellation of findings associated with tumors of the frontal lobe: optic atrophy in the ipsilateral eye, disc edema in the contralateral eye (due to increased ICP), central scotoma in the ipsilateral eye, and anosmia ipsilaterally