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The malignancy rate of pheochromocytoma is about 10 % . The malignancy rate of paraganglioma is close to 35%(1 ) .
What Is The Difference Between Paraganglioma & Pheochromocytoma?
Paraganglioma and pheochromocytoma are both neuroendocrine tumors arising from the chromaffin cells , which secrete catecholamine ( adrenaline and norepinephrine ) . They are rare tumors go on in 2 - 8 per 1 million persons per year and 0.1 % of hypertensive patients .
allot to WHO , these tumors are classified based on their localisation / origination , so they are relegate as adrenal bulb tumor or extra - adrenal paraganglia neoplasm . Pheochromocytoma is the term given to the chromaffin cell tumors plant in the adrenal secretor , which makes up nigh 85 % of all the tumors . The tumors outside of the adrenal gland are known as supererogatory - adrenal pheochromocytomas or paragangliomas , which constitute 15 % of all chromaffin cell tumors(1),(2 ) .
While most of these tumors are sporadic , approximately 35 % of these are familial in origin make them powerfully hereditary . Most of these tumors are found within the abdomen consist 98 % of all the tumor , including both phaeochromocytoma and paraganglioma . Paragangliomas can be found anywhere between the base of the skull to the pelvic region , admit the head and cervix region ( along the vagal and glossopharyngeal brass ; also known as carotid body and glomus jugulare body ) , thorax , urinary bladder bulwark , heart , mediastinum , the harmonium of Zuckerkandl ( around the origin of the subscript mesenteric artery ) . While pheochromocytomas are mostly likable , paragangliomas can be both charitable and parasympathetic . The parasympathetic paragangliomas do not secrete catecholamines and are mostly find in the head and neck opening region along the vagal and glossopharyngeal nerves(1),(2 ) .
Potential For Malignancy
Paragangliomas are more aggressive with a malignancy rate of around 35 % ; whereas , pheochromocytomas only show a 10 % malignancy rate . It is difficult to keep apart a benignant tumour from a malignant one . The risk factors for the metastatic disease let in tumor sizing of principal tumor > 5 cm ( in SDHB related tumors > 3.5 centimetre ) , extra - adrenal location , presence of SDHB ( succinate dehydrogenase fractional monetary unit gene ) mutations , jr. old age of initial diagnosing and lofty level of 3 - MT . The most common sites of metastasis let in the liver , lung , finger cymbals , and lymph node . The overall 5 - year survival of the fittest of malignant tumor is approximately 60%(1),(2 ) .
Clinical Features Of Paragangliomas And Pheochromocytomas
Headacheis the most coarse symptom afflicting about 90 % of the patients along withexcessive sweating(mostly truncal ) in about 60 - 70 % of the patient role , and pallor in around 27 % . The most vulgar triad of symptoms , which admit headache , shaking , and sweating found in patient stick out from hypertension should raise the alarm for these tumour . Other symptoms that the patients may plain of include anxiety , sluice , tremor , weakness , sickness , vomiting , truncation of breather , weight personnel casualty , fatigue , affright attack , and jumpiness . Other less common symptom include fever and constipation . Hypertension is mostly paroxysmal with instances of normal origin pressure , but can also be protract hypertension . Some patient may also go through hypotension ( due to increase spirit level of epinephrine there is excessive stimulation of beta - adrenoceptors ) , postural hypotension , or alternating hypertension and hypotension episodes(2 ) .
These signs and symptoms may pass in severe spells that may shop from several times a day to few time in a calendar month , their length may last from few transactions to several day at a prison term and with meter these fire may become more frequent and the severeness also increases with the increase in the size of the neoplasm . During an plan of attack , patient role may experience visual problems and utmost tiredness along with increased urination after an attack(1 ) .
Excess catecholamine may precede to cardiovascular complications , include severe high blood pressure , cardiac arrhythmias , acutemyocardial infarction , pulmonic dropsy , shock , andheart failure(2 ) .
Management Of Paragangliomas And Pheochromocytomas
The surgical removal of the tumor is the treatment of selection . However , it is important to manage the patients with medicine before the operating theater to see to it the levels of broadcast catecholamine , which may lead to hypertensive crisis and arrhythmias . The 5 - twelvemonth survival pace of benignant pheochromocytoma is greater than 95%(1),(2 ) .
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