Clinical trial: New Imaging Techniques in the Evaluation of Patients With Ectopic Cushing Syndrome (NCT00001849)

Eligibility criteria:
-  INCLUSION CRITERIA:

        All eligible patients are invited to participate in this protocol. Patients are adults with
        possible ectopic Cushing syndrome. Since both men and women are affected with ectopic
        Cushing syndrome, both sexes are studied. All ethnic and racial groups are at risk and will
        be included. Patients must be willing to return to the National Institutes of Health (NIH)
        Clinical Center for follow-up studies.

        EXCLUSION CRITERIA:

        Pregnant or lactating women. A pregnancy test is performed in women of childbearing
        potential (up to age 55) unless they have a history of hysterectomy.

        Children (age less than18) are excluded. Because ectopic ACTH secretion is rare in this age
        group, the likelihood of benefit is less and does not balance the risk of radiation.

        Patients taking medications that alter CYP3A4 activity will not be eligible for the
        mifepristone study, since this P450 system metabolizes mifepristone. Such participants
        would receive a clinical high dose (18 mCi) octreoscan (H-OCT) instead, if the standard 6
        mCi octreoscan (L-OCT) was negative. Patients with hypokalemia (K < 3.5 milliequivalent
        (mEq)/L) despite medical therapy with replacement or mineralocorticoid antagonists will
        also be excluded from the mifepristone studies.

        The presence of:

          -  severe active infection.

          -  clinically significantly impaired cardiovascular (e.g., history of abnormally low
             ejection fraction, the presence of moderate pulmonary fluid overload or leg edema, and
             blood pressure over 190/100), abnormal coagulation (partial thromboplastin time or
             prothrombin time elevated by 30 percent above the normal values), hematopoietic
             (hematocrit less than 30 percent, hemoglobin below 10 g/dl, white count below 3000
             K/microliter (UL), and platelets below 100,000 K/mm(3)), hepatic (liver enzymes
             elevated by 3-fold above normal values) or renal function (plasma creatinine level
             over 2.0).

          -  impaired mental capacity or markedly abnormal psychiatric evaluation that precludes
             informed consent.

          -  body weight over 136 kg, which is the limit for the tables used in the scanning areas.

          -  combined blood withdrawal, during the six weeks preceding the study, of greater than
             450 ml.

          -  known allergy to 111-indium pentetreotide or other somatostatin analogues.

          -  strong evidence for Cushing disease. This includes those with positive inferior
             petrosal sinus sampling or a lesion on pituitary MRI.
No condition on gender to be admitted to the trial.
No healthy subjects accepted to join the trial.
Subject must be at least 18 Years old.
Subject must be at most 90 Years

Patient description (medical language): A 47-year-old man comes to the office due to weight gain and fatigue.  He is not able to lift heavy objects or climb stairs. Family history is positive for DM type 2 and HTN in his father.  Blood pressure is 165/90 mm Hg and pulse is 85/min.  On physical examination, there is symmetric proximal muscle weakness of the upper and lower extremities.  Fasting plasma glucose level is 138 mg/dL and 24-hour urinary cortisol is twice the upper normal limit.  Further evaluation reveals that high-dose, but not low-dose, dexamethasone suppresses serum cortisol levels.  Serum ACTH levels are high-normal. This patient's findings are consistent with endogenous Cushing Syndrome.

Patient description (patient's own words): I'm a 47-year-old man. I went to the clinic because I had been gaining weight and had increased fatigue over the past few weeks. I was not able to lift heavy objects or even climb the stairs. My dad was diagnosed with type 2 diabetes and high blood pressure. During the exam, they took my blood pressure, which was 165/90 mm Hg, and my pulse was 85/min. They found out that I have weaknesses in both of my arms and legs. I also did a blood test on an empty stomach, and I had a glucose level of 138 mg/dL, and twice the upper normal limit of cortisol. I had high-normal levels of ACTH. The doctor said I suffer from endogenous Cushing Syndrome.

Does the patient match the trial criteria? 1