extra - adrenal pheochromocytomas ( eaps ) , more accurately known as paragangliomas , are rare tumours that account for 10 - 25% of pheochromocytomas .
some 53% of eaps occur in the organs of zuckerkandl , which are a collection of paraganglia situated anterolateral to the distal abdominal aorta between the origin of the inferior mesenteric artery and the aortic bifurcation .
the traditional treatment of a paraganglioma of organ of zuckerkandl ( poz ) involves open surgical excision , and only seven previous cases of laparoscopic resection have been reported [ table 1 ] .
we describe successful laparoscopic resection of a large poz in a young woman and review the previously reported cases .
a slender , 22-year - old woman with a body mass index ( bmi ) of 23 was being treated for hypertension for 2 years but it remained poorly controlled despite three antihypertensive drugs .
an abdominal ultrasound showed an 8 cm  7 cm mass on the left side of the abdomen .
a contrast - enhanced computed tomograph ( ct ) [ figure 1a ] confirmed a vascular tumour lateral to the aortic bifurcation and extending along the left common iliac artery .
haematological and biochemical investigations were normal except for grossly elevated plasma free normetanephrine and free metanephrine levels .
a metaiodobenzylguanidine ( mibg ) scan [ figure 1b ] showed intense uptake in the area corresponding to the mass identified on the ct scan , but no other areas of uptake were identified .
a work - up for multiple endocrine neoplasia type 2 ( men2 ) was negative .
her preoperative preparation included a high - salt diet and alpha blockade with prazosin for 2 weeks , followed by beta blockade with propranolol for 1 week .
( a ) ct scan showing a vascular tumour ( arrow ) adjacent to the aortic bifurcation ( b ) mibg scan showing increased uptake in the area corresponding to the tumour identified on the ct scan a laparoscopic excision was planned with the patient placed in the partial left lateral decubitus position .
a 10-mm port was placed in the right iliac fossa for the telescope using an open entry .
a 5-mm port was placed to the left side of this port , and another 5 mm port in the midline in the suprapubic area [ figure 2 ] .
a steep trendelenburg position allowed the small bowel and omentum to be moved away from the tumour , which was seen protruding through the descending and sigmoid mesocolon [ figure 3a ] .
a long horizontal incision was made in the anterior leaf of the mesocolon using an ultrasonic shears ( harmonic scalpel , ethicon endo - surgery , mumbai , mh , india ) .
the inferior mesenteric artery lying posterior to the tumour was carefully separated from it and preserved .
the mesocolic incision was closed with a running 2/0 polydiaxanone ( pds , johnson and johnson , mumbai , india ) .
the specimen was placed in a plastic bag and extracted through a 5-cm pfannenstiel incision that incorporated the incision made for the suprapubic 5-mm port . except for brief periods of hypertension during dissection of the tumour she remained stable throughout surgery .
the total operative time was 125 min and intraoperative blood loss was around 40 ml .
she was observed in the intensive care unit overnight , started on liquids and diet the day after surgery and discharged on post - operative day 4 .
the histopathology report confirmed the tumour to be a paraganglioma with no features of malignancy .
she remains normotensive and off her antihypertensive medication at the 18-month follow - up , at the time of writing , and her ct scan and biochemical tests , repeated 1 year after the surgery , were normal .
positions of the ports and the suprapubic 5-cm incision used for extraction of the specimen .
dotted circle denotes the position of the palpable tumour ( a ) large tumour protruding into the descending mesocolon ( b ) vessel entering the tumour from the inferior mesenteric artery being clipped
paraganglia are the main source of catecholamine production in the foetus and regress after 3 years of age .
failure of chromaffin cells at the site of paraganglia to regress leads to the development of paragangliomas .
the majority of the tumours occurring in the organ of zuckerkandl are benign and non - functional , but malignant and functional tumours are known to occur at this site . nearly half of the paragangliomas are familial and associated with neurofibromatosis type 1 , men2 , von hippel - lindau syndrome or hereditary paraganglioma syndrome .
the symptoms of paragangliomas may be related either to excessive catecholamine secretion or pressure symptoms due to a retroperitoneal mass .
headache , fainting and dizziness with hypertension are common with norepinephrine - producing tumours , whereas those secreting epinephrine tend to produce irritability , tachycardia , diarrhoea , excessive sweating , flushing and possibly hypertension .
yadav et al . reported a paraganglioma producing ureteral obstruction , whereas blecha et al .
plasma free metanephrines and urinary fractionated metanephrines carry the highest sensitivity ( 99% and 97% , respectively ) compared to the other plasma or urinary metabolites .
a ct scan is usually the first imaging test performed but may not identify lesions smaller than 2 cm
. a magnetic resonance imaging ( mri ) scan has higher sensitivity and specificity in detection of paragangliomas , which appear hyperintense on t2-weighted sequences . alpha blockade ( with phenoxybenzamine or prazosin ) followed by beta blockade ( with propranolol or a cardio - selective beta blocker ) as well as adequate hydration are crucial in the preoperative preparation of poz .
close intraoperative haemodynamic monitoring and control of hypertensive or hypotensive episodes that may occur by an experienced anaesthetist allows the surgery to proceed smoothly .
surgical resection is the treatment of choice for poz , and laparoscopic resection is suited for small ( < 7 cm ) tumours that show no radiological signs of invasion .
depending on their location , strategies utilised to approach the tumour included duodenal kocherisation as well as colonic mobilisation .
only thaper et al . described a mesocolic approach similar to ours , but they utilised three 10-mm ports , one sited at the umbilicus and the others on the left side of the abdomen . in our patient
we chose to place the 10-mm camera port in the right iliac fossa to allow direct , end - on visualisation of the tumour .
the original plan of colonic mobilisation to approach the poz was modified to a transmesocolic approach once it was apparent that this would provide early access to the tumour vasculature arising from the aorta and inferior mesenteric artery .
the left ureter and the inferior mesenteric artery are structures that need to be safeguarded during resection of a poz , although on occasion the latter may have to be resected . in rare instances of malignant poz invading the aorta or the inferior vena cava , open resection and reconstruction of these vessels as reported by dossett et al . may be warranted .
extraction of the specimen in a plastic bag via a low pfannenstiel incision resulted in a cosmetic scar in our young patient .
histopathological examination alone does not accurately differentiate benign and malignant paragangliomas , and the diagnosis of malignancy is based on the presence of local invasion and/or presence of metastases .
in a collective review of 135 cases of poz found 41% to be malignant . in view of this
, long - term follow - up with annual clinical , biochemical and radiological evaluation is mandatory in all patients with poz . in case of local recurrence
however , palliative therapy in the form of iodine-131 ( i)-mibg may have to be resorted to if the tumour is unresectable or metastatic .
in conclusion , poz is a rare tumour that can be resected safely laparoscopically by experienced surgeons .
adequate preoperative preparation , careful intraoperative management as well as post - operative care form the cornerstones of successful outcome in these patients .
 paragangliomas are catecholamine - secreting neuroendocrine tumours arising from chromaffin tissue at extra - adrenal sites . 
 the commonest site for a paraganglioma is the organ of zuckerkandl . 
 traditional treatment of paraganglioma of organ of zuckerkandl ( poz ) involves open surgical resection , and only a few cases of laparoscopic approach to this pathology have been reported . 
 we report the successful laparoscopic resection of a large poz in a 22-year - old woman and review the previous cases reporting a laparoscopic approach to this rare tumour . 
INTRODUCTION
CASE REPORT
DISCUSSION
CONCLUSION
Financial Support and Sponsorship
Conflicts of Interest