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A Mini Review of Paragangliomas with Presentation of Two Cases

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A Mini Review of Paragangliomas with Presentation of Two Cases REVIE\N ARTICLE turnours. MRI surpasses metaiodobenzylgua nid ine (MIBG) scanning in A mini review of detecting adrenal disease and non- functional paragangliornas. paragangliomas MISG has greater sensitivity and specificity than MRI in the with presentation detection of extra- adrenal tumours, although both are of two cases superior to CT. MRI is currently the 1. Paragangliomas E Kader investigation of choice, arising from the adrenal vvith MISG being used MBChB (UCT) medulla in cases of disease Department of Radiology, 2. Aortico-sympathetic University of Cape Town recurrence, metastases paragangliomas or equivocal MRI. 3. Parasympathetic and Surgical cure is possible Abstract visceroautonomic in up to 85°/() of cas s. paragangl iomas The recurrence rate is Paragangliomas are rare 4. Paragangliomas not betvveen 5 and 10 oir>, tu rnou rs of the othervvise specified. vvith a 5 year survival paragangl ion system, They occur sporadically rate of 80 lo 95%. vvhich may, in or in families, and have approximately 80<1<) of recognised associations cases, be hormonally vvith other diseases. The Introduction active vvith potentially majority are benign. Paragangliomas are rare tumours lethal consequences. In Ninety percent of arising from the paraganglion system. this mini revievv, the tumours are adrenal and The incidence inthe general population classification, pathology, 1OCYo are extra-adrenal. is 0.001-0.002 %, and 0.1 to 0.5 % in pathophysiology and Patients commonly those with hypertension.V Affected clinical management are present vvith individuals may be asymptomatic if described. An atternpt is hypertension or the tumour is non-functional, or can also made to evaluate p.arcrxvsrria.l crises and present with a broad spectrum of the relative advantages the laboratory diagnosis and disadvantages of the hinges on the symptoms in the case of functional most commonly used demonstration of tumours. These tumours carry a high diagnostic irriagi ng elevated catecholamine morbidity and mortality if discovered modalities, viz. levels. Initial incidentally. Diagnosis relies on a high Magnetic Resonance management involves clinical index of suspicion and appro- Imaging (MRI), stabilisation vvith alpha- priate biochemical evaluation. Subse- Computed Tornography blockade follovved by quent management includes (CT) and radionuclide preoperative tumour stabilisation of the patient and scintigraphy. localisation. preoperative evaluation prior to sur- Paragangl iomas are MRI has similar gical excision. Surgical cure rates of divided into four accuracy to CT in 66-85 % have been reported, given anatomical classes: diagnosis of adrenal early intervention. 2,3 to page 12 11 SA JOURNAL OF RADIOLOGY. November 1998 A mini revievv of paragangliomas vvith presentation of tvvo cases from page 11 This review attempts to address the radiograph confirmed pul- recommended nomenclature and clas- monary oedema. The ab- sification of these tumours, as well as dominal x-ray was normal. their basic pathological and pathophysi- The plasma noradrenaline ological features. Numerous biochemi- level was 16 500 (normal cal tests are currently employed in di- range 80-2 200) and urine agnosis, and their relative merits and NMA was 90.9 (normal demerits are compared. MRI, CT and range 0-5).A large,para-aor- nuclear medicine (NM) scanning have tic softtissuemass inthe left largely replaced more invasive tech- infrarenalareawasnoted on niques in preoperative tumour localisa- Figure 2: MIBG scan showing an area of increased uptake in the region ultrasound (US) (Figure 3) of the aortic bifurcation (arrows). tion. The relative advantages, disadvan- and CT (Figure 4). The tages and recommended application of The lesion was excised and was mass was surgically excised and histol- these three modalities are considered. histologically confirmed to be a para- ogy confirmed a paraganglioma. Case 1 ganglioma. A 20-year-old man presented with Case2 a brief history of lower abdominal pain A 19-year-old man presented with accompanied by nausea and vomiting. a history of progressive dyspnoea, loss He had been entirely well prior to this of weight and palpitations. He was ad- presentation and had no significant fam- mitted to hospital in pulmonary ily history. On examination the patient oedema secondary to malignant hyper- was apyrexial, normotensive and not tension. He had been investigated ayear tachycardie.Tenderness was noted in the earlier for lethargy, episodic headache, left lower abdominal quadrant. The palpitations and epigastric pain with chest radiograph was normal. The ab- nausea and vomiting. On examination dominal x-ray revealed a soft tissue den- he was tachycardie and hypertensive sity to the left of the umbilicus. This was (blood pressure was ]83/139 mmHg) confirmed on ultrasound. Catecho- with clinical evidence of cardiac fail- lamine assays were normal. The CT ure. Fundoscopy revealed grade 3 scan demonstrated a 5 em lesion with hypertensive retinopathy. A chest a central low den- sity at the aortic bifurcation (Fig- ure 1). On the ba- sis of the incon- clusive findings above, an 1123 metaicdobenzyl- guanidine (MIBG) scan was performed and revealed an area óf increased up- Figure 3a-c: Longitudinal (3aand b) and transverse(3c) take in the region ultrasound scans demonstrating an infra-renaI6.S by 2.9 cm Figure 1: Computed tomogram with intravenous contrast medium demonstrat- inhomogeneous lesion in the left para-aortic area. Therelationship of the aortic bifur- ing a rounded soft-tissue mass with central low density (arrow) anterior to the of the lesion (thick arrows) to the left kidney (curved arrows),aorta cation (Figure 2). lumbar spine. (thin arrows), and psoas muscle (arrowhead)can be appreciated. to page 13 12 SA JOURNAL OF RADIOLOGY- November 1998 A mini rev ievv of parngrmgf iorrias vvitl t presentation of tvvo cases from page 12 classes of paragan- and adrenaline in ratios greater than glia usually occur is physiologically normal, while extra- in the head and adrenal tumours tend to excrete neck and generally noradrenaline exclusively. Isolated stain negative for adrenaline secretion is rare. A wide chromaffin, while variety of secreted peptides have been tumours of the lat- documented including endogenous ter two groups oc- opioids, neuropeptide Y, chromogranin cur along the aorta, A, dopamine, somatostatin and in association with calcitonin. Their functional signifi- blood vessels or cance is dubious.v' visceral organs, and are usually chro- Pathology Figure 4: Computed tomogram (without intravenous contrast agent) of the lower maffin positive. The incidence of paragangliomas abdomen demonstrating an inhomogeneous, soft-tissue mass in the left para-aortic region (arrow). This classification in the general population is 0.001- is very similar to 0.002 % and 0.1 - 0.5 % in the hyper- the more recent WHO classification tensive population. 1·3 The commonly Anatomy of 1980, which defines four classes of affected age group is 25-35 years with The paraganglion system is part of paraganglia on an anatomical basis: I a median age of 37 -40 years." Ther the Amine Precursor Uptake and 1. Paragangliomas arising from the is a known familial predisposition in Decarboxylation (APUD) system (or adrenal medulla. Only these are re- l G % of cases. In these situations the "diffuse neuroendocrine system") ferred to as phaeochromocytomas. incidence of multiplicity is 35%,while which modulates the function of the 2. Aortico-sympathetic paraganglio- bilateral phaeochromocytomas may nervous and endocrine systems. Para- mas associated with the sympathetic occur in up to 50 % of individuals, com- ganglion cells are derived from neuro- chain. pared to la % in sporadic cases.I-3,5,9 ectoderm and are topographically and 3. Parasympathetic paragangliomas There is a well recognised association physiologically integrated with the (including chemodectomas) and with other diseases, notably multiple autonomic nervous system.I,3,4 visceroautonomic paragangliomas endocrine neoplasia type 2a & b Aggregations of cells associated with (found in association with small blood (MEN 2a & 2b), Von Hippel-Lindau the sympathetic nervous system vessels in organs). disease, neurofibromatosis, Cushing's (chromaffin cells) constitute the chro- 4.Paragangliomas not otherwise speci- disease and Carney's triad (extra-ad- maffin system and can conveniently fied. renal paraganglioma, gastric leiomy- osarcoma and pulmonary chon- be conceptualised as being adrenal Pathophysiology medullary or extra-adrenal. The ex- droma). tra-adrenal cell groups are called para- All paraganglion cells store Eighty to ninety percent of para- ganglia and occur in or near the sym- catecholamines and may therefore be gangliomas are adrenal and 10-20 % pathetic chain, near major blood ves- hormonally active. The hormones are are extra-adrenal. In the extra-adre- sels, or in various organs. In addition, not released secondary to neurologi- nal group, 80 % (8-16% of all para- paraganglion cells are also encoun- cal impulses, as the tumours are not gangliomas) are abdominal, occurring tered in association with innervated. Ninety percent of adrenal most commonly in the organ of parasympathetic tissue.!" tumours are active, while approxi- Zuckerkandl (para-aortic body) and Glenner and Grimley, in 1974, mately 50 % of extra-adrenal tumours renal hila. One to two percent occur classified paraganglia, and tumours and 1 % of parasympathetic tumours in the thorax, usually the posterior arising from them, into four classes, are hormonally
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