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Re: Carcinoid tumors, the cause of FF, palpitations, diarrhea,,,, Monday, 22-Jan-01 02:11:17: While rare, carcinoid & pheochromocytoma tumors are worth ruling out especially if your symptoms are not related to psychological stress. ClickMe-Below from: - http://www.carcinoid.org/FAQSUM.htm "Which symptoms need to be present to diagnose carcinoid syndrome. Carcinoid syndrome requires one or more of the following for laboratory confirmation of the diagnosis: increased blood serotonin with decreased blood tryptophan, increased chromogranin A and increased urine 5HIAA or indole-3-acetic acid (IAA). In spite of the symptoms, if these criteria are not present, carcinoid syndrome is not the diagnosis." "How high is the 5-HIAA and was the urine collected on a serotonin-free diet? If it was more than just slightly elevated, then confirm its significance by testing blood serotonin, which should be increased, blood tryptophan, which should be decreased, and chromogranin, A which should be increased. If one or two of these are clearly abnormal, you should check the liver for metastatic tumor – by CT-scan with contrast or MRI. If negative, do an Octreoscan to search for tumor foci not seen by the other imaging techniques." "The urine 5HIAA test for carcinoid is crude and can be strongly influenced by special diet and drugs. The patient must be on a special diet before and during the urine collection. Even then the test can miss up to 50% of the cases. It should not be relied on alone. Other markers for carcinoid tumor are blood serotonin, tryptophan, chromogranin A, pancreatic polypeptide and of course the OctreoScan." "Carcinoid crisis is characterized by abrupt flushing of face and sometimes upper body, usually severe fall in blood pressure and even bronchospasm with wheezing can (infrequently) occur. The attack may look like an anaphylactic attack. Diarrhea is an important part of carcinoid syndrome but is not usually simultaneous with the carcinoid crisis." "We would like to help you, but regulations do not allow me to answer case specific questions via the Internet. The statement on our website states we can answer “general” questions. I can state that the main features of carcinoid syndrome are flushing, diarrhea and a carcinoid tumor somewhere in the body which usually has spread to the liver and produces serotonin, chromogranin A and other substances and usually results in excretion in the urine of increased amounts of 5-HIAA, the breakdown product of serotonin. The designation “atypical” when applied to carcinoid tumor refer to its microscopic appearance, not its clinical behavior " "What other conditions are similar to carcinoid? Pheochromocytoma, Mast Cell Disease, Gastrointestinal Allergies, Vipoma, Medullary Carcinoma of the Thyroid, certain rare brain tumors and also certain rare neuropsychiatric disorders, to mention a few" Pheochromocytoma ClickMe-Below from: - http://www.pheochromocytoma.org/sys-tmpl/frequentquestions/ "There is a blood test available which tests Plasma Metanephrines and Catecholamines, which has been approved for diagnosis of pheochromocytoma." "Testing results indicate a sensitivity of 99% compared to 63% for VMA and 83% tom 85% for urinary or plasma catecholamines. " ClickMe - Below from: - http://www.his.com/~graeme/pheo.html Measurements of plasma concentrations of metanephrines appears to be the best test at present time for pheochromocytoma which usually presents with Hypertension. "Measurements of plasma concentrations of metanephrines (normetanephrine and metanephrine) provides a test that can reliably exclude the presence of a pheochromocytoma. Other currently available tests (e.g. plasma catecholamines, urinary metanephrines, urinary VMA) can result in false-negative results in some patients with the tumor (i.e. these tests do not reliably exclude a tumor). Thus, exclusion of pheochromocytoma typically requires multiple and repeated tests carried out with considerable expense. A missed diagnosis can have disastrous consequences for the patient. The advantage of plasma free metanephrines over other tests is that these measurements appear to reliably exclude a pheochromocytoma in suspected cases so that no further tests are necessary. Apart from avoiding a missed diagnosis, there are obvious cost-benefits of this for any health-care system. " Below From: http://www.mc.vanderbilt.edu/peds/pidl/endocr/pheochr.htm Endocrinology PHEOCHROMOCYTOMA The early diagnosis of pheochromocytoma is important, not only because it offers the possibilty of curing hypertension but also because unrecognized pheochromocytoma is a potentially lethal condition. Hypertensive crisis or shock or both leading to death have been precipitated by drugs, anesthesia, parturition, or surgery for unrelated conditions. Moreover, 8- 10% of the tumors are malignant. Pheochromocytoma most commonly arises from chromaffin cells of the adrenal medulla but may be found wherever chromaffin tissue is located. The tumor involves the right adrenal more often than the left, but many more tumors in children are bilateral or multiple. There is a 3:2 male preponderance and familial occurrance is common. It can be transmitted as an autosomal dominant trait. The tumor may be associated with such neurocutaneous syndromes as neurofibromatosis, von Hippel- Lindau disease, tuberous sclerosis, Sturge- Weber syndrome, and as a component of the multiple endocrine adenomatoses. The most common symptoms are headache, palpitations, and excessive and inappropriate perspiration. In a recent series of 76 patients with pheochromocytoma, all but three had one or more of these three symptoms, and 55 had at least two. This symptomatic triad in a hypertensive patient has a specificity of 94% and a sensitivity of 90%. The absence of all three symptoms in a hypertensive patient virtually ensured that pheochromocytoma was absent. Less commonly encountered symptoms include nervousness and anxiety, tremor, pallor, nausea, weakness, exhaustion, fatigue, chest or abdominal pains, and weight loss. Flushing is actually so rare that its presence casts doubt on the diagnosis of pheochromocytoma. In up to 50% of patients all of these symptoms are paroxysmal. Familial pheochromocytoma is associated with medullary carcinoma of the thyroid and adenoma or hyperplasia of the parathyroid glands and has been designated multiple endocrine neoplasia Type II or Sipple's syndrome. The coexistance of pheochromocytoma, thickened cranial nerves, alimentary track ganglioneuromatosis and marfanoid habitus constitutes multiple endocrine neoplasia Type III. The diagnosis of pheochromocytoma requires the demonstration of excessive catecholamine secretion. The most widely used procedure includes measurement of urinary catecholamines or their metabolites, vanillomandelic acid and total metanephrines. Of these, the urinary metanephrines provide a more sensitive clue to the presence of pheochromocytoma. Furthermore, plasma catecholamine measurements may be as reliable as the measurement of urinary metabolites. Determination of plasma catecholamines requires that the patient be in a fasting state and should rest comfortably in the supine position for at least 30 minutes before testing. The amounts of excreted free catecholamines and their metabolites vary depending on the levels of synthesizing and metabolizing enzymes within the tumor. Additional provocative testing may be done with a glucagon stimulation test. Glucagon is given as an IV bolus of 1.0- 2.0mg. A positive glucagon test requires a clear increase of at least three fold or over 2,000pg/ml in plasma catecholamines one to three minutes after drug administration. A simultaneous increase in blood pressure of at least 20mmHg should be present. A supression test uses the ability of clonidine, a centrally acting alpha- adrenergic agonist, to supress the release of neurogenically mediated catecholamines. Plasma catecholamines in patients with essential hypertension are supressed by clonidine, whereas they are unaltered in patients with pheochromocytoma. A normal clonidine supression tests consists of a fall of norepinephrine and epinephrine to a level below 500Pg/ml 2- 3 hours after the administration of 0.3mg of clonidine. Bravo et al have devised the following approach to patients with possible pheochromocytoma. Concentrations of total plasma catecholamines are measured after the patient is rested in a supine position for at least 30 minutes. Values over 2,000Pg/ml are considered pathognomonic of pheochromocytoma. Patients with values between 1,000 and 2,000Pg/ml receive a clonidine supression test. An abdominal CT scan is then performed on patients with clinical and biochemical features suggestive of pheochromocytoma. A recently developed technique for localization of neoplastic chromaffin tissues uses I-131 - metaiodobenzyl guanidine, a radioactive compound selectively taken up by adrenergic cells. Pheochromocytoma is cured by surgical removal of the tumor. Preoperativly patients should receive expansion of fluid volume for 12- 18 hours. Administration of an alpha- adrenergic blocking agent, such as phenoxybenzamine or prazosin for 7- 10 days before surgery will not only tend to re- expand the plasma volume but will also control hypertension and minimize blood pressure fluctuations during induction of anesthesia and throughout the operation. Pretreatment with a beta- blocking agent is not routinely necessary in the absence of arrythmias. Since it might induce a pressor response, beta- blockade should never be induced before alpha- blockade   Sadhelp