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1
Q

What is the cell of origin for vestibular schwannomas and acoustic neuromas (ANs)?

A

The Schwann cell of the myelin sheath is the cell of origin for ANs.

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2
Q

Which CN do ANs affect?

A

ANs affect CN VIII. Most affect the vestibular portion of the nerve.

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3
Q

In which anatomic region do ANs arise?

A

Most ANs are found in the cerebellopontine angle (CPA).

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4
Q

What are the most common presenting Sx, incidence, and what CNs are they associated with?

A

Hearing loss (95% of pts) and tinnitus (63%) → CN VIII; cochlear nerve unsteadiness/veering/tilting (61%) → CN VIII; vestibular nerve, facial paresthesia, or pain (17%) → CN V; facial paresis (6%) → CN VII. (Matthies C and Samii M, Neurosurg 1997)

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5
Q

What is the median age at Dx?

A

50 yrs

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6
Q

Most people with symptomatic ANs will present b/t what ages?

A

Most symptomatic pts are 30–50 yo.

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7
Q

What proportion of ANs are sporadic?

A

The majority (90%) are sporadic as well as unilat.

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8
Q

What % of ANs are bilat, and with what genetic abnormality are they associated?

A

10% are bilat and associated with NF-2, the tumor suppressor gene on chromosome 22.

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9
Q

What protein is abnl in NF-2 pts?

A

Merlin or schwannomin (involved in actin cytoskeleton organization)

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10
Q

What is the name of the anatomic layer of CN VIII that gives rise to most ANs?

A

The Obersteiner–Redlich zone (the junction b/t the central and peripheral myelin) gives rise to most ANs.

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11
Q

Subclinical ANs are present in what % of the general population?

A

Up to 1% (autopsy series) of the general population harbor subclinical ANs.

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12
Q

ANs account for what % of intracranial tumors?

A

5%–8% of intracranial tumors are ANs. Overall incidence is ∼1/100,000 person-yrs.

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13
Q

Apart from NF-2, what are 2 other risk factors that predispose to the development of ANs?

A

Loud noise exposure (ORR 13) and parathyroid adenoma (ORR 3.4), childhood exposure to low-dose RT (RR per Gy 1.14).

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14
Q

What are the Antoni A and Antoni B areas on histopathology?

A

Antoni A and B are zones of dense and sparse cellularity, respectively.

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15
Q

For what do ANs stain on immunohistochemistry?

A

Most ANs stain for S100.

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16
Q

How do bilat ANs fare after Tx when compared to unilat ANs?

A

Bilat ANs have similar failure rates to unilat lesions if treated adequately.