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Paper text:
paragangliomas of the adrenal medulla , the most common site of paragangliomas , are known as pheochromocytomas , whereas those located outside the adrenal gland as extra - adrenal paragangliomas ( eaps ) .
the diagnosis of pheochromocytomas and paragangliomas provide a potentially correctable cause of hypertension , and their removal can prevent hypertensive crises that can be lethal .
a 13-year - old girl was admitted with a history of fever , head ache , and vomiting of 1 month duration .
she did not give any history of significant illness in the past , other than occasional headache , which was relieved by medicines .
her pulse rate was 120 beats per minute , bp was 180/130 mmhg in the supine position and 140/110 mmhg in the upright position .
her blood investigations revealed a hemoglobin of 14.2 g% , total leukocyte count of 12,900 /mm with polymorphs 85% , lymphocytes13% , packed cell volume 45% .
her blood sugar , renal function tests , liver function tests , and serum electrolytes were within normal limits .
she had lymphocytic pleocytosis in the cerebrospinal fluid with protein content of 139 mg% and sugar of 92 mg% ( corresponding blood sugar 85 mg% ) .
twenty - four hour urinary vanilmandelic acid ( vma ) and urinary metanephrines levels were elevated .
ct abdomen with nonionic contrast showed a well - defined lobulated heterogeneously enhancing soft tissue mass lesion of size 4.3  3.4  3.7 cm in the right para - aortic location at the level of l3 vertebra with no necrosis or calcification .
minimal compression was noted in the inferior vena cava with preserved fat plane . there was no lymph node enlargement .
the possibility of aortosympathetic paraganglioma was entertained . with the clinical and investigation results a diagnosis of tuberculous meningitis with eap was made .
she was started on antituberculous drugs , - and -blockers , and calcium channel blockers .
the diagnosis of pheochromocytomas and paragangliomas provide a potentially correctable cause of hypertension . the first reported case of pheochromocytoma
alezais and peyron described extra - adrenal chromaffin tumors and called them paragangliomas in 1906 .
eaps are divided into 2 categories : those related to the parasympathetic system and those connected with the sympathetic system .
the former , usually nonchromaffin , lie in the head and neck , including the carotid body , jugulotympanic , and mediastinal and aorticopulmonary paraganglia .
the latter are chromaffin positive , associated with the peripheral sympathetic nervous system and secrete catecholamines .
they lie in the para - axial region of the trunk close to the paravertebral and prevertebral ganglia .
rarely , paragangliomas have been described in other unusual sites , such as the gallbladder , mesentery , kidney , and prostate .
eaps can be inherited as an isolated autosomal dominant trait or as part of the multiple endocrine neoplasia type ii syndromes , as well as with neurofibromatosis and von hippel 
germ line mutations in the succinate dehydrogenase ( sdh ) subunit genes sdh b , sdh c , sdh d , and sdh af2 predispose carriers to tumors of the paraganglia .
although eaps can be nonfunctional , the majority of eaps occurring below the diaphragm are functional with the symptoms related to the excessive secretion of catecholamines , namely , norepinephrine . in eaps ,
the lower levels of phenylethanolamine n - methyltransferase ( the enzyme responsible for the conversion of norepinephrine to epinephrine by methylation ) may lead to higher production of norepinephrine . commonly reported symptoms include hypertension ( paroxysmal and/or sustained ) , headaches , sweating , palpitations , anxiety , and tremors .
catecholamine crises can lead to heart failure , pulmonary edema , arrhythmias , and intracranial hemorrhage .
first , sporadic adrenal pheochromocytomas often affect patients in the 5th to 7th decades of life , with a slight female predilection .
eaps affect patients in the 2nd or 3rd decade of life , with genetic tumors having a male predilection .
the diagnosis is based on documentation of catecholamine excess by biochemical testing and localization of the tumor by imaging . in this patient the clinical scenario as well as biochemical evidence of raised urinary vma and metanephrines and also imaging evidence give unequivocal confirmation of the diagnosis .
pheochromocytomas and paragangliomas synthesize and store catecholamines , which include norepinephrine ( noradrenaline ) , epinephrine ( adrenaline ) , and dopamine .
elevated plasma and urinary levels of catecholamines and the methylated metabolites , metanephrines , are the cornerstone for the diagnosis .
urinary tests for vma , metanephrines ( total or fractionated ) , and catecholamines are commonly used for initial testing . among these tests ,
plasma tests include measurements of catecholamines , metanephrines , and chromagranin a , a secretory product of endocrine cells .
false - positive elevations occur due to stress , including venipuncture , medications ( eg , levodopa , labetalol , sympathomimetics ) .
t2-weighted magnetic resonance imaging with gadolinium contrast is somewhat better than ct for imaging extra - adrenal pheochromocytomas and paragangliomas .
a heterogeneous , hypervascular , retroperitoneal mass with areas of necrosis with typical clinical setting , is highly predictive of the presence of eaps .
of the paraganglionic tissues adjacent to the aorta , the organs of zuckerkandl are the most common site of involvement . the organs of zuckerkandl are located along the aorta , beginning cranial to the superior mesenteric artery or renal arteries and extending to the level of the aortic bifurcation or just beyond .
the organs of zuckerkandl are not often visualized radiologically unless they are involved by a pathologic process .
ct features of eap overlap those of other types of tumors found in the retroperitoneum .
specifically , tumors of neural or mesodermal origin and those of metastatic disease must be considered .
the close relationship of paragangliomas to the aorta in all instances may be of some value in differentiating these tumors from other primary retroperitoneal tumors .
tumors can also be localized using radioactive tracers , including i or i metaiodobenzylguanidine ( mibg ) , in - somatostatin analogs . however , there is a 10% false - negative rate with mibg scanning .
most malignant tumors show an enhanced glycolytic metabolism with increased uptake of deoxyglucose that can be visualized by pet using [ 18f]2-fluoro - d-2-deoxyglucose ( fdg ) .
oral prazosin or intravenous phentolamine can be used to manage paroxysms , while awaiting adequate -blockade .
-blockers can be added after starting -blockers , and increased as needed , if tachycardia persists .
because -blockers can induce a paradoxical increase in bp in the absence of -blockade , they should be administered only after effective -blockade .
other antihypertensives , such as calcium channel blockers or angiotensin - converting enzyme inhibitors , have also been used when bp is difficult to control with phenoxybenzamine alone .
blood pressure can be labile during surgery , particularly at the onset of intubation or when manipulating the tumor .
nitroprusside infusion is useful for intraoperative hypertensive crises , and hypotension usually responds to volume infusion .
although eaps can be nonfunctional , the majority of eaps occurring below the diaphragm are functional with the symptoms related to the excessive secretion of catecholamines , namely , norepinephrine . in eaps ,
the lower levels of phenylethanolamine n - methyltransferase ( the enzyme responsible for the conversion of norepinephrine to epinephrine by methylation ) may lead to higher production of norepinephrine . commonly reported symptoms include hypertension ( paroxysmal and/or sustained ) , headaches , sweating , palpitations , anxiety , and tremors .
catecholamine crises can lead to heart failure , pulmonary edema , arrhythmias , and intracranial hemorrhage .
first , sporadic adrenal pheochromocytomas often affect patients in the 5th to 7th decades of life , with a slight female predilection .
eaps affect patients in the 2nd or 3rd decade of life , with genetic tumors having a male predilection .
the diagnosis is based on documentation of catecholamine excess by biochemical testing and localization of the tumor by imaging . in this patient
the clinical scenario as well as biochemical evidence of raised urinary vma and metanephrines and also imaging evidence give unequivocal confirmation of the diagnosis .
pheochromocytomas and paragangliomas synthesize and store catecholamines , which include norepinephrine ( noradrenaline ) , epinephrine ( adrenaline ) , and dopamine .
elevated plasma and urinary levels of catecholamines and the methylated metabolites , metanephrines , are the cornerstone for the diagnosis .
urinary tests for vma , metanephrines ( total or fractionated ) , and catecholamines are commonly used for initial testing . among these tests ,
plasma tests include measurements of catecholamines , metanephrines , and chromagranin a , a secretory product of endocrine cells .
false - positive elevations occur due to stress , including venipuncture , medications ( eg , levodopa , labetalol , sympathomimetics ) .
t2-weighted magnetic resonance imaging with gadolinium contrast is somewhat better than ct for imaging extra - adrenal pheochromocytomas and paragangliomas .
a heterogeneous , hypervascular , retroperitoneal mass with areas of necrosis with typical clinical setting , is highly predictive of the presence of eaps . of the paraganglionic tissues adjacent to the aorta , the organs of zuckerkandl are the most common site of involvement .
the organs of zuckerkandl are located along the aorta , beginning cranial to the superior mesenteric artery or renal arteries and extending to the level of the aortic bifurcation or just beyond .
the organs of zuckerkandl are not often visualized radiologically unless they are involved by a pathologic process .
ct features of eap overlap those of other types of tumors found in the retroperitoneum .
specifically , tumors of neural or mesodermal origin and those of metastatic disease must be considered .
the close relationship of paragangliomas to the aorta in all instances may be of some value in differentiating these tumors from other primary retroperitoneal tumors .
tumors can also be localized using radioactive tracers , including i or i metaiodobenzylguanidine ( mibg ) , in - somatostatin analogs . however , there is a 10% false - negative rate with mibg scanning .
most malignant tumors show an enhanced glycolytic metabolism with increased uptake of deoxyglucose that can be visualized by pet using [ 18f]2-fluoro - d-2-deoxyglucose ( fdg ) .
oral prazosin or intravenous phentolamine can be used to manage paroxysms , while awaiting adequate -blockade .
-blockers can be added after starting -blockers , and increased as needed , if tachycardia persists .
because -blockers can induce a paradoxical increase in bp in the absence of -blockade , they should be administered only after effective -blockade .
other antihypertensives , such as calcium channel blockers or angiotensin - converting enzyme inhibitors , have also been used when bp is difficult to control with phenoxybenzamine alone .
blood pressure can be labile during surgery , particularly at the onset of intubation or when manipulating the tumor .
nitroprusside infusion is useful for intraoperative hypertensive crises , and hypotension usually responds to volume infusion .
a 13-year - old , previously asymptomatic girl was admitted with features of tuberculous meningitis . 
 she was found to be hypertensive and further investigations revealed an extra - adrenal paraganglioma . 
 tuberculous meningitis and paraganglioma could be chance associations . 
 paraganglioma is a very rare and potentially lethal cause of secondary hypertension . 
 we are reporting a very rare disease , which has come to light in a most unexpected manner .