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intimately 10 - 15 % of paragangliomas are asymptomatic(1 ) .
Delay in diagnosis can cause life-threatening cardiac complications and may even lead to sudden death(1 ) .
Is Paraganglioma A Serious Condition?
Paragangliomas can be symptomless as well as symptomatic ; asymptomatic ranging from 10 - 15 % that can be diagnose apropos through imaging done for other lawsuit or in autopsy cause . The symptoms of paragangliomas can be extremely varied owe to the production of different catecholamines ( epinephrine , norepinephrine , and dopamine ) secretion , non - secernment of catecholamine by HNPGLs , emplacement of the tumor mass , the involvement of other variety meat when associated with other hereditary syndrome and tenacious - term picture to high levels of circulating catecholamines(1 ) .
Hypertension in advance cases of PCCs and sPGLs is either determine as lengthened or paroxysmal . The classical triad of symptoms , namely , spells of quiver , worry , and sweating ( excessive sweating ) along with hypertension are observe . If these symptom are present then paraganglioma should be suspected and biochemical examination should be carried out . These convulsion can last from few minutes to hours or even day and the oftenness also varies . These spells can either go on impromptu or be actuate through some body process , symptomatic procedures , sure food ( caffein , aminoalkane - containing foods , sure cheeses ) , drug ( metoclopramide , glucocorticoids , glucagon ) .
Other associated symptom admit weakness , nausea , vomiting , anxiety , and hyperglycemia . Due to the atypical nature of the tumour , there can be a delay in diagnosing and this might run to serious cardiac complications , such as myocardial infarction , arrhythmias , center failure , cerebrovascular accidents , and even sudden demise . Therefore , an immediate diagnosis is necessary . Head and neck opening paragangliomas can present with painless the great unwashed , hearing loss , trouble in swallowing and speech , tinnitus , cranial nerve palsy , and hoarseness of voice(1 ) .
Can Paragangliomas Be Reversed?
The diagnosis of paragangliomas can be done with biochemical testing by mensurate the plasma grade of metanephrines and/or 24 - hour urine excretion of fractioned metanephrines . prescribed biochemical examination can be confirmed by imaging , including MRI , CT scan , MIBG ( I - metaiodobenzylguanidine ) scintigraphy , and PET scan(1 ) .
Since most of the paragangliomas are benign , the consideration can be reversed by surgically excising the tumor mickle and the patients can be symptom - free and lead a normal life . Benign paragangliomas are also affiliate with an excellent prospect . There are minor postoperative knottiness including hypotension and hypoglycemia , which can be well managed and subside in a few day . Regular one-year follow - up with biochemical testing and blood line pressure command is necessary to supervise patient for the opening of a local recurrence or metastases . However , 10 % of phaeochromocytoma and 10 - 20 % of paragangliomas are malignant and their management is a little scrap slippery . Malignant neoplasm are managed by operative resection of the primary tumor and the regional lymph nodes . radiation syndrome therapy is also given to slow up down the progress of the remaining disease . Combination chemotherapy ( cyclophosphamide , Oncovin , and dacarbazine ) is also used in MIBG - negative patients .
These patient can be managed palliatively with conventional actinotherapy , arterial embolization , and chemoembolization for painful bone metastases and radiofrequency ablation for liver metastases . Catecholamine deductive reasoning inhibitor can be used to thin out the signs and symptoms related to excess catecholamine secretions . Their prognosis is also misfortunate with a 5 - yr survival rate of around 20 - 50%(1 ) .
Paraganglioma is a rare neuroendocrine tumor arising from the neural crest cells along with the autonomic nervous system ( large-hearted and parasympathetic anxious system ) . The associated likable paragangliomas of the adrenal glands are do it as pheochromocytomas ( PCCs ) ; whereas , outside the adrenal glands they are know as sympathetic paragangliomas ( sPGLs ) . The parasympathetic eccentric paragangliomas are more common in the head and cervix region ; therefore , they are termed as head and neck paragangliomas ( HNPGLs ) .
loosely , people of all age group are affected , but the great incidence is seen in individuals of 40 - 50 eld mathematical group . It as affects the male and female population(1 ) .
Although most of the paragangliomas are sporadic , one - third of neoplasm are hereditary and associated with inherited syndrome . Generally , most of the paragangliomas are benign tumor ; about 10 - 15 % can be malignant that spread to distant sites devoid of chromaffin tissue , such as ivory , lungs , liver , and lymph nodes(1 ) .
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