Managing your symptoms: clinical syndromes and the drugs to treat them Laurence Katznelson, MD
Carcinoid symptoms Flushing (peripheral vasomotor symptoms) Diarrhea Bronchoconstriction (ashma/wheezing, with bronchial carcinoids Valvular heart disease Fatigue Low blood pressure Cushing s syndrome Excessive steroid production by the adrenal glands due to factors produced by the carcinoid tumor 2
Management of symptoms Avoid precipitating factors Symptomatic control Endocrine Somatostatin analogs Chemotherapy Surgery 3
Management of symptoms Precipitated by: Stress (i.e. emotional, physical) Infection Foods Avoidance of precipitating factors 4
Triggers: Foods containing high levels of amines (tyramine and dopamine) Aged cheeses (cheddar, Camembert, Stilton) Alcohol Smoked, salted or pickled fish or meat (herring, salami, sausage, corned beef, bologna, pepperoni) Any spoiled protein foods (chicken liver) Yeast extracts and Brewers yeast, hydrolyzed proteins Beans, sauerkraut, shrimp paste, some soybean 5
Triggers: Foods containing lower levels of amines (tyramine and dopamine) Caffeine containing drinks, coffee (in large amounts), soda Chocolate (in large amounts) Some nuts (peanuts, coconuts, brazil nuts) Some pizzas, raspberries, banana, avocado 6
Carcinoid diarrhea Non-specific diarrhea treatment Opioids Codeine tincture of opium Imodium cyproheptadine (serotonin antagonist) 7
Targeted medical therapy Somatostatin analogs Flushing and diarrhea resolve in the majority Octreotide Available as Sandostatin, subcutaneous injections given every 6-8 hours Sandostatin LAR, intramuscular (buttock) depot injection every 4 weeks, sometimes every 3 weeks 8
Somatostatin analog therapy Side effects Diarrhea, bloating (usually only during the first few days after the monthly injection) Gallstones (usually not symptomatic) Uncommon hair loss, dizziness Hyperglycemia: if someone has diabetes mellitus, need to follow glucose closely 9
Somatostatin analog therapy In addition to octreotide, there is lanreotide, not currently available to patients in the US Based on biochemistry, this should be very similar in effect and side effect profile Lanreotide is administered as deep subcutaneous injection in the buttock, at the same intervals Pasireotide (SOM 230) Novartis compound currently under investigation A novel somatostatin analog, and may be more effective. 10
Carcinoid: Cushing s syndrome Cushing s syndrome is defined as excess cortisol (steroid) production Associated with weight gain, full/red face, weakness, osteoporosis, hypertension, and diabetes The carcinoid tumor produces ACTH or CRH: these are both hormone chemicals that travel in the body to the adrenal glands, which then make more cortisol 11
Cushing s syndrome 12
Cushing s syndrome: associated problems Weakness Bone loss: osteoporosis Change in body habitus: more central fat Immunocompromised (from excess steroids) Hypertension Diabetes mellitus Elevated lipids Risk for clotting 13
Cushing s syndrome: treatment Management Treatment of the carcinoid tumor itself, such as surgery Somatostatin analogs very useful to reduce the ACTH/CRH, leading to lowering of the cortisols We sometimes need to use medications which can reduce the adrenal gland activity Rarely, surgery to remove the adrenal glands may be necessary to normalize the cortisol levels 14
Q & A 15
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