phechromocytomas are rare cathecholamine secreting tumors derived from the chromaffin cells of the embryonic neural crest .
although these tumors are similar in origin , the clinical manifestations , prognosis and management differ and occurrence of pheochromocytoma in patients with hypertensive symptoms is less than 0.5% . while pheochromocytomas and abdominal paragangliomas are catecholamine - producing tumors of the sympathetic nervous system , head and neck paragangliomas are non - secreting tumors of parasympathetic origin .
recent developments in clinical and molecular research on these tumor forms have significantly clarified their genetic backgrounds and challenged the view of pheochromocytoma as the 10% rule tumor .
it has been known for some time that pheochromocytomas present as a component of the familial syndromes multiple endocrine neoplasia type 2 , von hippel - lindau disease and , rarely , neurofibromatosis type 1 .
germline mutations have been identified in these diseases in , respectively , the proto - oncogene ret , and the tumor suppressor genes vhl and nf1 .
more recently , germline mutations in the genes encoding succinate dehydrogenase subunit d ( sdhd ) and subunit b ( sdhb ) have been described in patients with pheochromocytomas .
the current definition of a malignant pheochromocytoma is the presence of metastases , although elegant attempts have been made to outline histopathological risk factors for the malignant course of these tumors .
we report a case of adrenal phechromocytoma in a 25-year - old man who presented with hypertension , palpitations and excessive sweating that was managed by left adrenalectomy after medical control of symptoms , and who is free from recurrence one year after surgery .
we also highlight the techniques adopted in pre- and peri - operative management of hypercathecolaminemia , the difficulty in predicting malignancy or otherwise , and strategies for improving patient compliance with follow up in a developing country .
a 25-year - old man was referred by nephrologists with recurrent episodes of palpitation and headache of two years duration .
he also complained of excessive sweating that meant he had to wash 67 times a day .
he was started on amlodipine 10 mg die , -methyldopa 250 mg three times a day , and ranitidine 300 mg at night for two weeks , all in tablet form .
the following tests were perfomed : serum electrolytes urea and creatinine ( s / e / u / cr ) , chest x - ray , postero - anterior view ( cxr , pa view ) , serum protein , lipid profile , urinalysis , full blood count ( fbc ) , human immune deficiency virus screening ( hiv screening ) , hepatitis b surface antigen screening ( hbsag ) , hepatitis c virus screening ( hcv ) , abdominal ultrasound scan and 24 h urine assay . the results are listed in table 1 . 

table 1results of tests performed.resultsnormal ranges / e / u / crna136 mmol / l(135145 mmol / l)k4.6 mmol / l(3.55.0 mmol / l)hco328 mmol / l(2428 mmol / l)cl98 mmol / l(97108 mmol / l)urea3.1 mmol / l(2.58.5 mmol / l)cr2.6 mmol / l(44.2194.5 mmol / l)ca22.6 mmol / l(2.22.8 mmol / l)po40.8 mmol / l(11.018.0 mmol / l)serum proteintotal74 g / l(6285 g / l)albumin48 g / l(3550 g / l)globulin26 g / l(2335 g / l)lipid profilecholesterol3.7 mmol / l(<5.1 mmol / l)hdl1.7 mmol / l(>1.5 mmol / l)ldl1.9 mmol / l(<2.6 mmol / l)vldl0.1 mmol / l(>0.77 mmol / l)tg0.3 mmol / l(<1.7 mmol / l)urinalysis*appearanceyellow and clearspecific gravitynormalreactionacidproteinnilsugarnilketonenilbilenildepositswhite blood cells ( wbc ) 24 /hpf;red blood cells ( rbc ) 01/hpfcastsnilfull
blood countrbc4.3810/l(4.36.210/l)pcv37%(4052%)mchc32.0 g / dl(3135 gm / dl)mcv83.6 fl(82102 fl)platelets23910/l(14045010/l)wbc6.110/l(4.110.910/l)differential wbc countneutrophil39%(3580%)eosinophil02%(07%)basophils01%(02%)lymphocytes55%(2050%)monocytes03%(212%)screenshiv1 and 2negativehbsagnegativehcvnegative*cultures yielded no bacterial growth . 
  cultures yielded no bacterial growth .
the abdominal ultrasound ( performed on may 4 , 2010 ) showed : a well delineable hypoechoic solid avascular mass measuring about 985866 mm in the left upper quadrant of the stomach bed .
it has 3 cysts , each about 3 cm to 5 cm in diameter in the right upper pole .
the right kidney measures 11041 mm while the left measures 10744 mm in size ; both kidneys are normal in echopattern .
ultrasound showed the spleen , liver , pancreas , gallbladder , etc . to be normal .
cxr performed on 05/05/2010 showed normal radiological findings ; the magnetic resonance imaging ( mri ) on 26/5/2010 ( figure 1 ) showed a t2 weighted image of an ovoid left adrenal mass measuring 965765 mm with bright signal intensity in its upper aspect suggesting a cystic component . 

figure 1magnetic resonance imaging showing the left pheochromocytoma , with tumor outlined by yellow arrows . 
 
impression : left adrenal solid / possible cystic mass , phechromocytoma . further results as follows : i ) pre - operative urine : vma 11 mg/24 h ( 27 mg/24 h ) ; ii ) pre - operative metanephrine : 126 g/24 h ( 2496 g/24 h ) ; iii ) pre - operative normetanephrine : 415 g/24 h ( 7575 g/24 h ) ; iv ) post - operative metanephrine : 31 g/24 h ( 2496 g/24 h ) ; v ) post - operative normetanephrine : 81 g/24 h ( 75375 g/24 h ) .
these results suggested phechromocytoma and left adrenalectomy was programmed , while antihypertensives were continued until the patient was normotensive ( 110/60 mmg ) .
several pre - operative meetings were also held with the anesthetist to discuss any possible problems and find solutions .
pre - operativemeasures adopted were : intravenous normalsaline 1 l 6-hourly and liberal oral intake of fluids24 h before surgery for volume expansion .
open left adrenalectomy was then performed under epidural anesthesia ( later combined with general anesthesia with cuffed endotracheal tube , and muscle relaxation because of slow onset of the former ) via an extended left sub - costal incision
. a much - enlarged left adrenalgland measuring 1065 cm ( figure 2 ) andweighing 200 g was removed .
no apparent involvement of regional lymph nodes , liver , bowel , pancreas or spleen was noted .
a significant spike in blood pressure and pulse rate was noted on intra - operative palpation of the tumor ( maximal at 220/120 mmhg and pulse rate of 196 bpm ) .
this was managed by increasing the rate of infusion of propofol until excision was complete .
immediately post left adrenalectomy , bp and pr were 90/54110/70 mmhg and 84 bpm . at a joint postoperative meeting , it was agreed that the patient did not require admission to the intensive care unit and returned , therefore , to the surgical ward .
post - operative recovery was uneventful , and the patient 's vital signs were assessed half hourly for the first 6 h and hourly for the next 18 h , with the patient remaining normotensive .
histology [ hematoxylin & eosin ( h&e ) ] showed a nested pattern , zellballen appearance ( figure 4 ) , nuclear pleomorphism and hyperchromasia as shown by bizarre tumor giant cells ( figure 5 ) . 

diagnosis was phechromocytoma with a pheochromocytoma of the adrenal gland scaled score ( pass ) of 8 , ( table 2 ) . 

table 2pheochromocytoma of the adrenal gland scaled score ( pass).criterion assessedscorevascular invasion ( 1)-capsular invasion ( 1)-peri - adnexal adipose tissue involvement ( 2)-large nests or diffuse growth ( 2)-focal or confluent necrosis ( 2)-high cellularity ( 2)2tumor cell spindling ( 2)-cellular monotony ( 2)-increased mitotic figures ( > 3/10hpf ) ( 2)2atypical mitotic figures ( 2)2profound nuclear pleomorphism ( 1)1hyperchromasia ( 1)1total8 

phechromocytomas are rare cathechola - mine secreting tumors derived from the chro - maffin cells of the embryonic neural crest .
approximately 85% arise from chromaffin cells in the adrenal medulla , and 15% arise from chromaffin tissue in extra - adrenal sites extending from the neck to the pelvis , although most are found intra - abdominally .
common presenting features are hypertension , palpitations and sweating , which our patient had for two years before a diagnosis was made .
it has been shown that phechromocytoma is a rare tumor in black africans with similar symptomatology to other races . however , laboratory and radiological facilities for diagnosis are limited in sub - saharan africa
. a high index of suspicion remains the most important factor in determining whether a correct early diagnosis can be made .
the need for pre - operative control of blood can not be overemphasized , as cardiovascular complications such as congestive cardiac failure , hypertensive encephalopathy and pulmonary edema are well known , and it has been shown that pre - operative patient preparation has accounted for the most significant reduction in peri - operative mortality .
the use of  blockade using phenoxybenzamine or prazosin to reduce blood pressure and facilitate volume expansion is well established , but calcium channel blockers such as amlodipine can achieve a similar reduction without the risk of orthostatic hypotension .
access to the gland was obtained using an abbreviated chevron incision , which has been judged to provide good superior and lateral access to the adrenal gland , and gave excellent exposure that allowed controlled dissection .
it has been stated that , wherever possible , phechromocytoma should be removed using a laparoscopic approach , to achieve less postoperative pain , faster recovery and better cosmesis , unless the tumor is large as was the case in our patient .
we did note that the open approach afforded the patient safe and complete tumor resection .
there was no evidence of capsular or vascular invasion by the tumor , but considering the pass score of 8 , we counseled the patient on the need for long - term follow up .
this we have done by phone calls to remind the patient of clinic appointments and intermittent home visits to reaffirm earlier discussions regarding compliance with follow up .
this was greatly facilitated by the widespread availability of cell phones in nigeria , with the availability of global system for mobile telephony ( gsm ) mobile telephones since 2001 . at his last hospital visit ,
urine assay for metanephrine and normetanephrine were normal and repeat ultrasound showed no evidence of recurrence .
we would like to perform a postoperative magnetic resonance imaging or computerized tomography , but this is not yet possible due to the cost ( $ 450$1000 ) .
i - labeled meta - iodobenzylguanide scintigraphy and positron emission tomography using f fluorodopamine are not currently available in our setting . in conclusion , pheochromocytoma management is challenging and is made more so in countries with limited resources .
it can , however , be diagnosed and treated effectively , with a high index of suspicion especially amongst primary care physicians .
we recommend the following guidelines to aid diagnosis and treatment in developing countries . in settings with few resources ,
all newly diagnosed hypertensive patients under the age of 35 years be screened with the following simple easily available tests : i ) urine metanephrine ; ii ) urine normetanephrine ; iii ) urine vma ; iv ) abdominal ultrasonography to detect presence of adrenal and or other retroperitoneal swellings ; v ) genetic testing ( where available ) for germline mutations in ret proto oncogene , vhl tumor suppressor gene , nf-1 tumor suppressor gene , and b and d subunits of mitochondrial succinate dehydrogenase sdhb and sdhd , under the following conditions : i ) positive family history ; ii ) clinical evidence of syndromic disorder e.g. vhl and men 2 .
treatment : i ) determine total number of swellings and trigger factors ; ii ) perform abdominal , mri scanning or ct scan if mri is not available to localize tumor properly ; iii ) commence pre - treatment with calcium channel blockers ; iv ) avoid  blockers if possible ; v ) commence volume replacement on admission 2448 h before surgery , and assess adequacy of same with urine output monitoring ; vi ) establish contact and have a patient - centered discussion with an anesthetic team to anticipate problems and offer solutions to them proactively and prepare for same ; vii ) anticipate possible intensive care unit admission pre - operatively and prepare for same . in conclusion , the role of adequate pre - operative preparation is vital to reduce perioperative morbidity and mortality , and is safely achieved with calcium channel blockers and volume expansion using normal saline .
 pheochromocytomas are rare tumors that present a diagnostic challenge in developing countries . 
 they occur in the adrenal gland and as paragangliomas along the sympathetic chain . 
 clinical features are usually those of sustained or paroxysmal hypertension and complications thereof . 
 surgical extirpation remains the mainstay of treatment and is greatly facilitated by accurate pre - operative tumor localization . 
 pre - operative medical management with antihypertensive medication has led to significant reductions in peri - operative mortality . 
 determination of malignancy is difficult in the absence of obvious metastases . 
 we present a case of left adrenal phechromocytoma that was stabilized . 
 adrenalectomy had a good outcome and the patient has so far been followed up for a year . 
Introduction
Case Report
Discussion