Inclusion Criteria

- INCLUSION CRITERIA:

Patients must have histologically confirmed alveolar soft part sarcoma. Pathology should
be confirmed at the Laboratory of Pathology, National Institutes of Health.

Patients must have measurable disease, defined as at least one lesion that can be
accurately measured in at least one dimension (longest diameter to be recorded) as greater
than or equal to 20 millimeters with conventional techniques or as greater than or equal
to 10 millimeters with spiral CT scan.

Patients must have metastatic alveolar soft part sarcoma that is not curable by surgery.

Patients who have surgically resectable tumors with metastasis will be considered on a
case by- case basis.

Any prior therapy must have been completed greater than or equal to 4 weeks prior to
enrollment on protocol and the participant must have recovered to eligibility levels from
prior toxicity. Patients should be at least 6 weeks out from nitrosoureas and mitomycin C.
Prior radiation should have been completed greater than or equal to 4 weeks prior to study
enrollment and all associated toxicities resolved to eligibility levels. Patients must be
greater than or equal to 2 weeks since any investigational agent administered as part of a
Phase 0 study (also referred to as an early Phase I study or pre-Phase I study where a
sub-therapeutic dose of drug is administered) at the PI's discretion, and should
haverecovered to eligibility levels from any toxicities.

Any degree of prior treatment is allowed, including other anti-angiogenic treatments
(e.g., VEGFR2 inhibitors or bevacizumab). Patients with no prior therapy are eligible,
provided they have metastatic disease that is not curable by surgery.

BSA greater than or equal to 0.63 square meter.

ECOG performance status less than or equal to 2 for adults, Karnofsky performance status
greater than or equal to 50% for pediatric patients greater than 10 years of age, and
Lansky performance status greater than or equal to 50 for pediatric patients less than or
equal to 10 years of age.

Life expectancy of greater than 8 weeks.

Patients must have normal organ and marrow function as defined below:

- absolute neutrophil count greater than or equal to 1,500/microliter

- platelets greater than or equal to 100,000/microliter

- total bilirubin less than 1.5 times institutional upper limit of normal

- AST(SGOT)/ALT(SGPT) less than or equal to 2.5 times institutional upper limit of
normal

- creatinine within normal limits based on age as follows:

Age (Years) Maximum Serum Creatinine
(milligrams per deciliter)

less than or equal to 5 0.8

5 less than age less than or equal to 10 1.0

10 less than age less than or equal to 15 1.2

greater than 15 1.5

OR

creatinine clearance greater than or equal to 60 milliter/min for adults or greater than
or equal to 60 milliter/min/1.73m2 for children with creatinine levels above institutional
upper

limit of normal.

QTc must be less than 500 msec.

Pediatric patients: Normal left ventricular function with ejection fraction greater than
55% or shortening fraction greater than or equal to 7%.

At present, the potential of AZD2171 for clinically significant drug interactions
involving the CYP isozymes is unknown. However, studies of the agent in rats indicated
possible suppression of CYP1A that may be of biological significance. A list of drugs that
may interact with the cytochrome P450 system is included.

Eligibility of patients receiving any medications or substances known

to affect or with the potential to affect the activity of PK of AZD2171 will be determined
following review of their case by the Principal Investigator. Efforts should be made to
switch patients with brain metastases who are taking enzyme-inducing anticonvulsant agents
to other medications one week prior to starting therapy.

AZD2171 has been shown to terminate fetal development in the rat, as expected for a
process dependent on VEGF signaling. For this reason, women of child-bearing potential
must have a negative pregnancy test prior to study entry. Women of child-bearing potential
and men must agree to use adequate contraception (hormonal or barrier method of birth
control; abstinence) prior to study entry and for the duration of study participation.
Should a woman become pregnant or suspect she is pregnant while participating in this
study, she should inform her treating physician immediately.

Ability to understand and the willingness to sign a written informed consent document.

Patients should not be receiving any other investigational agents.

Prior therapy with anti-angiogenic agents is permitted.

EXCLUSION CRITERIA:

Patients with clinically significant illnesses which would compromise participation in the
study, including, but not limited to: active or uncontrolled infection, uncontrolled
diabetes, uncontrolled hypertension, symptomatic congestive heart failure, unstable angina
pectoris, myocardial infarction within the past 6 months, uncontrolled cardiac arrhythmia;
or psychiatric illness/social situations that would limit compliance with study
requirements.

Patients may not be receiving any medication that may markedly affect renal function
(e.g., vancomycin, amphotericin, ibuprofen, pentamidine).

Patients who are unable to swallow tablets.

Mean QTc greater than 500 msec (with Bazett's correction) in screening electrocardiogram
or history of familial long QT syndrome.

Greater than +1 proteinuria on two consecutive dipsticks taken no less than 1 week apart.

Pregnant women are excluded from this study because AZD2171 is a VEGF inhibitor with known
abortifacient effects. Because there is an unknown but potential risk for adverse events
in nursing infants secondary to treatment of the mother with AZD2171, breastfeeding should
be discontinued if the mother is treated with AZD2171.

HIV-positive patients on combination antiretroviral therapy are ineligible because of the
potential for PK interactions with AZD2171.

Hypertension not controlled by medical therapy (hypertension defined as systolic blood
pressure greater than 150 mmHg or diastolic pressure greater than 90 mmHg despite optimal
medical management).

Adult patients with hypertension not controlled by medical therapy (hypertension defined
as systolic blood pressure greater than 150 millimeters of mercury or diastolic pressure
greater than 90 millimeters of mercury despite optimal medical management). Pediatric
patients must have BP WNL for age. NOTE: blood pressure within the upper limit of normal
is defined as: blood pressure less than or equal to the 95th percentile for age, height,
and gender, and measured, and not be receiving medication for treatment of hypertension.