Inclusion Criteria:

- Age ≥ 3 years and body surface area ≥ 0.5 m2

- Meets diagnostic criteria for NF2

- At least one volumetrically measurable and ≥ 0.5 cc NF2-related brain or spinal tumor
(schwannoma, ependymoma, meningioma - histological confirmation not required) with
radiographic evidence of progression (either as unequivocal progression on
conventional MRI, or a >10% volume increase by 3D volumetrics) over the past ≤12
months, designated as the primary target tumor OR Volumetrically measurable and ≥ 0.5
cc VS with ipsilateral progressive hearing loss over the past ≤12 months, designated
as the primary target tumor

- Progressive Hearing Loss Criteria for Enrollment: Audiogram showing drop in pure tone
average (PTA) of 10dB HL at ≥ 2 nonconsecutive or consecutive frequencies or drop in
speech discrimination score (SDS) below the 95% critical difference threshold,
compared to previous audiogram ≤ 1 year prior.

- Karnofsky/Lansky performance status (PS) 50-100%. Note: Patients who are unable to
walk because of paralysis, but who are up in a wheelchair, will be considered
ambulatory for the purpose of assessing the performance score.

- Adequate bone marrow function as shown by: ANC ≥ 1.5 x 109/L, Platelets ≥ 100 x
109/L, Hb > 9 g/dL

- Adequate liver function as shown by:

1. serum bilirubin ≤ 1.5 x ULN

2. ALT and AST ≤ 2.5x ULN

- INR ≤ 1.5. (Anticoagulation is allowed if target INR ≤ 1.5 on a stable dose of
warfarin or on a stable dose of LMW heparin for >2 weeks at time of enrollment.)

- Adequate renal function: serum creatinine ≤ 1.5 x ULN

- Fasting serum cholesterol ≤300 mg/dL OR ≤ 7.75 mmol/L AND fasting triglycerides ≤ 2.5
x ULN. NOTE: In case one or both of these thresholds are exceeded, the patient can
only be included after initiation of appropriate lipid lowering medication.

- Fully recovered from acute toxic effects of any prior chemotherapy, biological
modifiers or radiotherapy

- Any neurologic deficits must be stable for ≥ 1 week

- Able to provide signed informed consent (or consent by parent/legal guardian for
minors)

Exclusion Criteria:

- Patients currently receiving medical anticancer therapies or who have received
medical anticancer therapies within 4 weeks of the start of study drug (including
chemotherapy, antibody based therapy, etc.)

- Radiation therapy to a study target tumor within 1 year prior to enrollment, or any
radiation therapy within 4 weeks prior to enrollment.

- Patients who have had a major surgery or significant traumatic injury within 4 weeks
of start of study drug, patients who have not recovered from the side effects of any
major surgery (defined as requiring general anesthesia) or patients that may require
major surgery during the course of the study

- Prior treatment with any investigational drug within the preceding 4 weeks

- Patients receiving chronic, systemic treatment with corticosteroids or another
immunosuppressive agent. Topical or inhaled corticosteroids are allowed.

- Patients should not receive immunization with attenuated live vaccines within one
week of study entry or during study period. Close contact with those who have
received attenuated live vaccines should be avoided during treatment with everolimus.
Examples of live vaccines include intranasal influenza, measles, mumps, rubella, oral
polio, BCG, yellow fever, varicella and TY21a typhoid vaccines.

- Uncontrolled brain or leptomeningeal metastases, including patients who continue to
require glucocorticoids for brain or leptomeningeal metastases

- Other malignancies within the past 3 years except for adequately treated carcinoma of
the cervix or basal or squamous cell carcinomas of the skin.

- Patients who have any severe and/or uncontrolled medical conditions or other
conditions that could affect their participation in the study such as:

1. Symptomatic congestive heart failure of New York heart Association Class III or
IV

2. unstable angina pectoris, symptomatic congestive heart failure, myocardial
infarction within 6 months of start of study drug, serious uncontrolled cardiac
arrhythmia or any other clinically significant cardiac disease

3. severely impaired lung function as defined as spirometry and DLCO that is 50% of
the normal predicted value and/or 02 saturation that is 88% or less at rest on
room air

4. uncontrolled diabetes as defined by fasting serum glucose >1.5 x ULN (Note:
Optimal glycemic control should be achieved before starting trial therapy.)

5. active (acute or chronic) or uncontrolled severe infections

6. liver disease such as cirrhosis or severe hepatic impairment (Child-Pugh class
C).

Note: A detailed assessment of Hepatitis B/C medical history and risk factors must be done
at screening for all patients. HBV DNA and HCV RNA PCR testing are required at screening
for all patients with a positive medical history based on risk factors and/or confirmation
of prior HBV/HCV infection.

- A known history of HIV seropositivity

- Impairment of gastrointestinal function or gastrointestinal disease that may
significantly alter the absorption of everolimus (e.g., ulcerative disease,
uncontrolled nausea, vomiting, diarrhea, malabsorption syndrome or small bowel
resection)

- Patients with an active, bleeding diathesis

- Female patients who are pregnant or breast feeding, or adults of reproductive
potential who are not using effective birth control methods. Adequate contraception
must be used throughout the trial and for 8 weeks after the last dose of study drug,
by both sexes. (Females of childbearing potential must have a negative urine or serum
pregnancy test within 7 days prior to administration of everolimus)

- Male patient whose sexual partner(s) are WOCBP who are not willing to use adequate
contraception, during the study and for 8 weeks after the end of treatment

- Patients who have received prior treatment with an mTOR inhibitor (e.g., sirolimus,
temsirolimus, everolimus).

- Patients with a known hypersensitivity to everolimus or other rapamycins (e.g.,
sirolimus, temsirolimus) or to its excipients

- History of noncompliance to medical regimens

- Patients unwilling to or unable to comply with the protocol