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Neuro 2 > CNS Tumors (Gianani) > Flashcards

CNS Tumors (Gianani) Flashcards

1
Q

TUMORS OF NEUROGLIAL CELLS

A

A. Tumors of Glial Cells:

Astrocytic tumors:

Astrocytoma
Glioblastoma multiforme

Oligodendroglioma

Ependymoma - choroid plexus papilloma

B. Neuronal Tumors
Ganglioglioma
Gangliocytoma
Central neurocytoma

C. Embryonal Tumors
Medulloblastoma

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2
Q

Most common tumor in the brain

A

metastatic

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3
Q

tumor that most often metastasizes to the brain

A

melanoma

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4
Q

glioblastoma multiforme is another name for

A

astrocytoma grade 4

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5
Q

Ependymoma

A

choroid plexus papilloma

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6
Q

GERM CELL TUMORS

A

Teratoma

Craniopharyngioma

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7
Q

TUMORS OF CRANIAL

AND SPINAL NERVES

A

Schwannoma
Neurofibroma

can occur peripherally too

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8
Q

The terms “intra-axial” and “extra-axial,”

A

used in radiological descriptions, mean “in brain or spinal cord tissue” and “extrinsic to brain” respectively.

For instance, astrocytoma and oligodendroglioma are intra-axial; meningioma and Schwannoma are extra-axial.

The term anaplasia describes the cellular atypia and loss of differentiation that are associated with malignant tumors.

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9
Q

OLIGODENDROGLIOMA: genetic alteration

A

1p and 19q codeletion

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10
Q

ATYPICAL TERATOID-RHABDOID TUMOR genetic alteration

A

Loss of 22q

particularly bad prognosis- see it in infants

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11
Q

MENINGIOMA: genetic alteration

A

Loss of 22q

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12
Q

NF1

A

NF1/17q

NS tumors: Plexiform neurofibroma, MPNST, optic and other gliomas

Other tumors: Pheochromocytoma, GIST

cafe au lait spots

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13
Q

NF2

A

NF2/22q- the gene is merlin

Vestibular and PN shwannoma, meningioma, other brain tumors
(*acoustic schwannoma)

not associated with other tumors

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14
Q

Li- Fraumeni

A

TP53/17p

Astrocytoma

Breast carcinoma, bone and soft tissue sarcoma

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15
Q

tuberosclerosis

A

SEGA

Renal AMLs, lung LAM, ** cardiac rhabdomyoma

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16
Q

VHL

A

VHL/3p

Hemangioblastoma

Renal cell carcinoma, pheochromocytoma

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17
Q

Von Recklinghausen neurofibromatosis (VRNF - Neurofibromatosis type 1-NF1)

A

one of the most common genetic disorders, is autosomal dominant and is caused by mutations of a gene on chromosome 17q that encodes a protein called neurofibromin.

Neurofibromin is involved in control of cell proliferation and acts as a tumor supressor.

  • have a variety of tumors, including * bilateral optic nerve astrocytomas, and plexiform neurofibromas and malignant peripheral nerve tumors.
  • café au lait spots of the skin, axillary and inguinal freckles, dysplasia of the sphenoid wing and other skeletal abnormalities, fibromuscular dysplasia of arteries, and other lesions.
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18
Q

DDx of intracranial mass lesion

A

Infections

  • Cerebral abscess
  • Toxoplasmosis
  • Tuberculosis

Hemorrhage

  • Hematoma
  • Hemorrhagic metastasis

Neoplasms

  • Primary
  • Metastatic
19
Q

Classification- grades of tumors, etc.

A

Astrocytic Tumors

  • Pilocytic Astrocytoma, WHO Grade I
  • Diffuse Astrocytoma, Grades II, III, IV
  • Other (PXA, SEGA)

Oligodendroglial Tumors can have a variety of grades

Ependymal Tumors can have a variety of grades

Choroid Plexus Tumors
- Papilloma, Carcinoma

Neuronal and Glioneuronal Tumors
- Neurocytoma, Ganglioneurocytoma, Ganglioglioma

20
Q

3 tumors in the embryonal category

A

medulloblastoma, PNET, atypical TERATOID-RHABDOID TUMOR

21
Q

Meningioma

A

gets a big chunk of the brain tumor pie (MOST COMMON primary non-malignant tumor of CNS)

Dural-based, extra-axial tumor in adults…complete excision…cure
~25 to 30% of CNS tumors.
Arise from meningothelial cells of arachnoid.
More common in women
Most common genetic abnormality…loss of chromosome 22 (22q)…loss of function of protein merlin.
Multiple in NF2 and post radiation
50% sporadic tumors have mutation of NF2 gene
Majority have good prognosis
>90% WHO I (~5% II, ~2% III)
Many histologic types

22
Q

meningiomas and invasion

A

Meningiomas can be seen to invade bone and even muscle…does not mean it is malignant

majority are benign but that some can look and behave malignant.

23
Q

how meningiomas look on histology

A

In transitional meningiomas, tumor cells are arranged in whorls with hyalinized and calcified centers that are called psammoma (sand) bodies because they resemble tiny grains of sand.

24
Q

Gliomas

A

fairly common group of primary brain tumors, include astrocytomas, oligodendrogliomas, and ependymomas.

These tumor types have characteristic histologic features that form the basis for the classification.

  • It is no longer thought that these tumors derive from their specific, mature cell types (astrocytes, oligodendrocytes, and ependymal), but rather that they arise from a progenitor cell that preferentially differentiates down one of the cellular lineages.

Many of the tumors typically occur in certain anatomic regions within the brain, with characteristic age distribution and clinical course.

25
Q

grade 3 meningioma example

A

anaplastic meningioma

26
Q

The two major categories of astrocytic tumors are:

A
  1. infiltrating astrocytomas
    • diffuse astrocytoma (grade II / IV)
    • anaplastic astrocytoma (grade III / IV)
    • glioblastoma (grade IV / IV)

2) more localized astrocytomas, of which the most common are the pilocytic astrocytomas. (BETTER PROGNOSIS)

27
Q

Pilocytic Astrocytoma, WHO I

A

Most frequent glial tumor in children

Cerebellum
- Thalamus, hypothalamus, * optic nerve

Well circumscribed

Solid and cystic

Increased intracranial pressure, cerebellar signs

Slow growth

Overall good prognosis

28
Q

Diffuse Astrocytoma, WHO II

A 
Ill-defined, infiltrating
Any site in CNS
> supratentorial (F,T)
Seizures, headaches or focal signs
Tendency to progress to higher grade
Survival ~5-7 years
29
Q

Glioblastoma (GBM) WHO IV

A 
Most frequent glial tumor in adults
Highly malignant
Seizures, headaches, focal deficits
Supratentorial
Survival ~1 year
30
Q

primary vs 2ndary glioblastoma

A

can arise denovo or secondary to astrocytomas

31
Q

best prognosis for glioblastoma

A

None of the genetic characteristics have specific prognostic significance except for IDH mutations (proneural type tumors)…better prognosis

32
Q

Oligodendroglioma

A 
Ill-defined, infiltrating
Supratentorial, cortical and white matter
Frontal in 50-65%
Most common in 4th-5th decades
Long history of headaches, seizures
Frequently calcified
WHO II or III
Prognosis 6 years II
   (better prognosis than other gliomas)
33
Q

histological look of glioblastoma

A

foci of necrosis with pseudo-palisading of malignant nuclei and endothelial cell proliferation

34
Q

Oligodendroglioma gene mutations and prognosis

A

IDH1/IDH2 gene mutations in 90% and portend a better prognosis.

Deletions of chromosome 1p, together with * 19q seen in 80%…with these deletions, patients have consistent long lasting response to chemo and radiation…not so for the others.

35
Q

oligodengroglioma histologic appearance

A

fried egg

positive for GFAP protein

36
Q

Medulloblastoma, WHO IV

A

Malignant tumor of children
70% under 15 years of age

Posterior fossa, 75% in the vermis

Ataxia and signs of > IP

WHO IV

Tendency to disseminate along subarachnoid space

Prognosis

  • 50-70% at 5 years
  • (30% in the 1960’)
37
Q

Medulloblastomas are thought to arise from

A

stem cells located in the subependymal matrix and the external granular layer (EGL) of the cerebellum.

38
Q

Medulloblastoma histology

A

Classic medulloblastoma (the majority) is a highly cellular tumor composed of diffuse masses of small, undifferentiated oval or round cells.

Wright rosettes- groups of tumor cells arranged in a circle around a fibrillary center

39
Q

Medulloblastoma and malignancy

A
  • highly malignant tumor.

It infiltrates and destroys brain tissue and tends to seed the subarachnoid space and spread along the walls of the ventricles.

Leptomeningeal dissemination occurs more frequently in medullablastoma than any other BT

The CSF shows high protein and low glucose, and contains tumor cells. CSF cytology is used to monitor the spread of the tumor. Extracranial metastases occur rarely, usually after operation or shunting.

Treatment combines resection, to reduce the tumor mass and decompress the fourth ventricle, shunting of the lateral ventricles, radiation of the tumor bed and the entire neuraxis, and intrathecal chemotherapy.

40
Q

Primary CNS Lymphoma-PCNSL

A

PCNSL is typically a diffuse large B-cell lymphoma (DLBCL).

The tumors that arise in immunocompromised patients are EBV-positive.

peripheral

Meningeal spread is very common, and some PCNSLs arise in the subarachnoid space.

Corticosteroids induce apoptosis in PCNSL cells that may be so profound that the tumors disappear (vanishing lymphoma).

41
Q

METASTATIC TUMORS to BRAIN

A

majority
men- most commonly primary carcinoma of the lung
women- carcinoma of the breast

highest rate of metastasis- melanoma

42
Q

Ependymoma

A

Slow growing tumors, children and young adults

Hydrocephalus, >IP, ataxia

3-9% of CNS tumors
Intraventricular, spinal cord

Well circumscribed, but can disseminate

WHO I, II and III

Prognosis
60%(5y) and 48% (10y) II
55%(5y) and 26% (10y) III

43
Q

tumors that form rosettes in the brain

A

retinoblastoma
ependymoma
medulloblastoma

44
Q

Schwannoma

A

Other names in books:
Neurilemoma, neurinoma, Vestibular or Acoustic Schwannoma

> > in Peripheral nerves, but in CNS > Vestibular branch of 8th

4-6 decades

Solitary (sporadic)

Bilateral in NF2

Benign

Neuro 2 (15 decks)

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