Case No. :	78
Month :	June
Year :	2005
Contributor :	Suvarna Barhate

Other Cases

A 18 year old lady presented with history of non colicky dull aching abdominal pain in abdomen since one month. On and off,she complained of palpitations, headaches and episodes of profuse sweating.She had presented with rashes over hands and legs On clinical examination,she had pallor, vitals were stable.Systemic examination was unremarkable.Serial BP monitoring detected episodic hypertension.Biochemistry revealed raised urinary VMA levels.

RADIOLOGICAL Examination:

Ultrasound abdomen revealed mixed echogenic mass in the region of right adrenal gland, with hypoechoic thrombus in the Right renal vein and the Suprarenal IVC showing vascularity on Doppler imaging.

Fig. 1	Fig. 2

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CT Abdomen confirmed the presence of right adrenal pheochromocytoma, with retroperitoneal and intratumoral hemorrhage and the presence of right renal vein thrombus with extension in the IVC.

Fig. 5	Fig. 6

Fig. 7

In order to confirm the presence of a tumoral thrombus , MR was done which showed no enhancement of the thrombus post contrast.

Fig. 8	Fig. 9
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With these imaging findings in mind, the decision was taken to operate the patient; IVC gram was performed to know the exact extent of the thrombus which confirmed the presence of thrombus along the right lateral wall of transrenal and suprarenal IVC.

Fig. 12

Follow up:

Patient underwent Right Nephrectomy with total excision of the tumor.Post-op, the patient recovered well and catecholamine levels were reduced.Rashes resolved on tenth post op day.

Fig. 13

Histopathology confirmed the tumor to be a benign pheochromocytoma,with fibrin thrombus in the renal vein.

DISCUSSION:

Phaeochromocytomas are catecholamine-producing tumours that arise from the paraganglion cells anywhere in the autonomic nervous system. Phaeochromocytomas originate in the adrenal medulla in 90% of cases; most extra-adrenal phaeochromocytomas arise in the paravertebral sympathetic ganglia, in the organ of Zuckerkandl (near the aortic bifurcation) or, rarely, in the bladder. About 90% of adrenal medullary phaeochromocytomas are functional but only 50% of sympathetic and 1% of parasympatetic tumours produce excess catecholamines . Ninety per cent of phaeochromocytomas occur sporadically, the remaining 10% are associated with neuroectodermal disorders such as neurofibromatosis, tuberous sclerosis, von Hippel-Lindau syndrome or multiple endocrine neoplasia syndromes. Multiple phaeochromocytomas occur in up to 10% of sporadic cases, but in as many as 30% when associated with a neuroectodermal syndrome . Approximately 10% of phaeochromocytomas are malignant.
Creutzfeldt-Jakob disease can occur sporadically - from exposure to contaminated products such as growth hormone extracts from pituitary glands of cadavers or rarely, it can be genetically inherited. The disorder is rare, occurring in about 1 in a million people. The average age at onset of symptoms is the late 50s - though it has been noticed from the second to the seventh decades.

Histologically, the cells are arranged in rounded clusters separated by endothelial lined spaces. Like the pheochromocytomas associated with MEN2, those associated with VHL are often multiple and ectopic; approximately 50-80% are bilateral .Although generally considered benign, VHL associated pheochromocytomas can metastasize both locally to nodes and to distant organs. Many pheochromocytomas in VHL are asymptomatic and do not elevate serum catecholamine levels .

When symptoms occur they can be varied and include, periodic or sustained hypertension, headaches, palpitations, episodic sweating, and anxiety attacks; such symptoms may be incorrectly attributed to anxiety or depression. Pheochromocytoma can cause such life threatening conditions as hypertensive crisis, myocardial infarction, cardiac failure, stroke and metastatic disease.

Laboratory tests for pheochromocytoma include serum and urinary norepinephrine (NE), epinephrine (E), and urinary vanillylmandelic acid (VMA). The 24-hour urine collection may also include metanephrine and dopamine but these are less sensitive markers. Urinary NE is more sensitive than urinary E and VMA .

Imaging tests include CT, MRI and the metaioodobenzylguanidine test (MIBG). CT is often performed with intravenous contrast because the kidney must also be evaluated. Pheochromocytomas typically enhance after contrast media although small portions of the tumor may remain low density . CT is excellent for evaluating the adrenals and organ of Zuckerkandl but is less ideal for investigating other ectopic sites where MRI is superior.

Pheochromocytomas, usually (95%) demonstrate high signal intensity on T2 weighted MRI, however, some lesions may be only slightly hyperintensive or even hypointense .

MIBG is about 95% sensitive for pheochromocytoma and is 100% specific but may not detect very small lesions. When an adrenal lesion is discovered by CT, an MRI is usually performed. If the lesion is small, asymptomatic and catecholamines are normal, the patient is generally followed with CT at yearly intervals at a minimum. If symptoms or elevated catecholamines develop, the MIBG is performed prior to surgery to exclude additional sites.

Before surgical removal the patient should have adequate adrenergic blockade beginning two weeks before surgery. Adrenalectomy is the preferred technique although this approach requires subsequent adrenal replacement therapy for bilateral disease .Enucleation of the tumor, partial adrenalectomy or bench surgery with autotransplantation into the thigh or arm have been used as methods of preserving adrenal function and deserve attention.

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