SOM230 Ectopic ACTH-producing Tumors

Inclusion Criteria:

- Written informed consent obtained prior to any screening procedures

- Male or female patients aged 18 years or greater

- Confirmed non-pituitary ectopic-ACTH secreting tumor

- Well differentiated, and low or intermediate grade (WHO classification G1-2)
neuroendocrine tumor

- Tumor size increase < 10% in 6 months prior to screening on CT or MRI

- Mean 24-hour urinary free cortisol level of at least 1.5 x the upper limit of the
normal range, and a morning plasma ACTH level of > 5 ng/L

Exclusion Criteria:

- Patients with highly malignant ACTH-secreting tumors, i.e. small-cell lung carcinomas,
medullary thyroid carcinomas, and pheochromocytomas

- Patients with poorly differentiated neuroendocrine tumors (WHO classification G3)

- Patients with >10% increase of tumor size in 6 months prior to screening by CT or MRI

- Patients with Cushing's syndrome due to pituitary ACTH secretion

- Patients with hypercortisolism secondary to adrenal tumors or nodular (primary)
bilateral adrenal hyperplasia

- Patients who have a known inherited syndrome as the cause for hormone over secretion
(i.e. Carney Complex, McCune-Albright syndrome, MEN-1)

- Patients with a diagnosis of glucocorticoid-remedial aldosteronism (GRA)

- Patients who have undergone major surgery within 1 month prior to screening

- Patients with known gallbladder or bile duct disease, acute or chronic pancreatitis
(patients with asymptomatic cholelithiasis and asymptomatic bile duct dilation can be
included)

- Diabetic patients whose blood glucose is poorly controlled as evidenced by HbA1C >8%

- Patients who have clinically significant impairment in cardiovascular function or are
at risk thereof, as evidenced by

- Congestive heart failure (NYHA Class III or IV), unstable angina, sustained
ventricular tachycardia, clinically significant bradycardia, high grade AV block,
history of acute MI less than one year prior to study entry

- QTcF >450 msec at screening

- History of syncope or family history of idiopathic sudden death

- Risk factors for Torsades de Pointes such as uncorrected hypokalemia, uncorrected
hypomagnesemia, cardiac failure

- Concomitant disease(s) that could prolong the QT interval such as autonomic
neuropathy (caused by diabetes or Parkinson's disease), HIV, cirrhosis,
uncontrolled hypothyroidism, concomitant medication(s) known to increase the QT
interval

- Patients with liver disease or history of liver disease such as cirrhosis, chronic
active hepatitis B and C, or chronic persistent hepatitis, or patients with ALT or AST
more than 2 x ULN, serum creatinine >2.0 x ULN, serum bilirubin >1.5 x ULN, serum
albumin < 0.67 x LLN at screening

- Patients with any ongoing or planned anti-neoplastic therapy

- Has been treated with radionuclide at any time prior to study entry

- Is likely to require any additional concomitant treatment to pasireotide for the tumor

- Patients who have any current or prior medical condition that can interfere with the
conduct of the study or the evaluation of its results, such as

- History of immunocompromise, including a positive HIV test result (Elisa and
Western blot). An HIV test will not be required, however, previous medical
history will be reviewed

- Presence of active or suspected acute or chronic uncontrolled infection

- History of, or current alcohol misuse/abuse in the 12 month period prior to
screening

- Female patients who are pregnant or lactating, or are of childbearing potential and
not practicing a medically acceptable method of birth control. If a woman is
participating in the trial then one form of contraception is sufficient (pill or
diaphragm) and the partner should use a condom. If oral contraception is used in
addition to condoms, the patient must have been practicing this method for at least
two months prior to screening and must agree to continue the oral contraceptive
throughout the course of the study and for 3 months after the study has ended. Male
patients who are sexually active are required to use condoms during the study and for
three month afterwards as a precautionary measure (available data do not suggest any
increased reproductive risk with the study drugs)

- Patients who have participated in any clinical investigation with an investigational
drug within 1 month prior to screening or patients who have previously been treated
with pasireotide

- Known hypersensitivity to somatostatin analogues

- Patients with a history of non-compliance to medical regimens or who are considered
potentially unreliable or will be unable to complete the entire study

- Patients with presence of Hepatitis B surface antigen (HbsAg)

- Patients with presence of Hepatitis C antibody test (anti-HCV)