|LETTER TO EDITOR
|Year : 2011 | Volume
| Issue : 2 | Page : 260-262
Bilateral cystic pheochromocytoma: An incidental finding and a rare case entity
S Gayathri, BP Baruah, S Kumar, P Garg
Department of Radiodiagnosis, PGIMER, Ram Manohar Lohia Hospital, New Delhi, India
|Date of Web Publication||11-Jul-2011|
Department of Radiodiagnosis, PGIMER, Ram Manohar Lohia Hospital, New Delhi
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Gayathri S, Baruah B P, Kumar S, Garg P. Bilateral cystic pheochromocytoma: An incidental finding and a rare case entity. Indian J Cancer 2011;48:260-2
Reports of cystic pheochromocytomas are sparse in literature. Cases of bilateral pheochromocytomas have been described in association with multiple endocrine neoplasia syndrome. Only 5.7% of incidentally discovered adrenal tumors are cystic. About one-third of pheochromocytomas have some cystic component.  However, bilateral cystic pheochromocytomas are extremely rare in patients without any overt symptomatology. We report a case of incidental synchronous "bilateral" "cystic" pheochromocytoma(s) in a young adult male.
A 19-year-old male presented to the Out Patient Department of our hospital with vague pain in abdomen and occasional headaches. On examination, no abdominal or neurological abnormality was found. The patient was referred for ultrasound examination of the abdomen.
On ultrasonography, a well-defined cystic mass with multiple thick septae was seen adjacent to segment 7 of liver. The imaging appearance prompted a differential diagnosis of either a hydatid of liver or an adrenal mass. On careful analysis, this complex mass was found to be separate from the liver with the presence of the classical hyperechoeic retroperitoneal fat in between them.
Doppler ultrasonography revealed that the mass was highly vascular with flow signals in the septations (low resistance flow). A smaller lesion was seen on the left side. It was predominantly cystic with peripheral vascularity [Figure 1] and [Figure 2].
|Figure 1: Thick-walled, complex mass with multiple thick vascular septa and hypo to an-echoic spaces. Retroperitoneal fat (arrow) separates the mass from the adjacent liver|
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Contrast-enhanced CT of the abdomen revealed bilateral cystic adrenal masses. Right-sided lesion was more than 6 cm in size with enhancing septae in it and on the left side, two cysts were seen in the region of the adrenal gland [Figure 3] and [Figure 4].
|Figure 3: Axial images and coronal reformat of contrast-enhanced CT scans through the upper abdomen showing the bilateral cystic complex adrenal masses|
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|Figure 4: Axial images and coronal reformat of contrast-enhanced CT scans through the upper abdomen showing the bilateral cystic complex adrenal masses|
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Urine for vanillyl mandelic acid (VMA) done in view of the possibility of pheochromocytoma was strongly positive. Later plasma catecholamines and metanephrines were also found to be elevated. Surgical and pathological correlation followed 3 weeks later and confirmed the diagnosis to be of bilateral pheochromocytomas beyond any doubt.
Traditionally known as the "10% tumor" (though the rule is no longer fool proof): pheochromocytomas are known to have the following characters: bilateral disease is present in approximately 10% of patients; 10% are malignant; 10% arise in childhood; 10% recur after resection; 10% present without hypertension.  Thirty two percent of pheochromocytomas are reported to be cystic.  Twenty five percent are familial in origin.  Fifteen percent are located in chromaffin tissue outside the adrenal. 
The typical imaging characteristics of a pheochromocytoma includes a round, well-circumscribed, homogeneous soft-tissue mass, measuring over 3 cm, enhancing heterogenously (caution needs to be exercised while administering contrast in these patients as it may trigger malignant hypertension).
Cystic lesions in the adrenal can be of the following types: parasitic cysts, epithelial cysts, cystic adenoma, trans-glandular retention cysts, pseudocysts (including pheochromocytoma and neuroblastoma), lymphangiomatous cysts, hemorrhagic cysts and necrotic cysts. 
Differential diagnosis of incidental adrenal masses (%) includes adenoma (51%), metastatic cancer (31%), adrenal cancer (4%), cyst (4%), pheochromocytoma (4%), hyperplasia (2%), lipoma (2%), myelolipoma (2%). 
In a nut shell - the differentials for bilateral adrenal masses which are cystic, multiloculated, and septated, includes
The cystic pheochromocytoma typically exhibits areas of low attenuation, with hounsfield units in the range of 5-15 and rim enhancement on contrast administration.  Incidental adrenal lesions with these features discovered on CT scanning are an indication for catecholamine assays to screen for pheochromocytoma.
- Bilateral adrenal hydatid cysts
- Adrenal carcinoma
| » References|| |
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|2.||Lee TH, Slywotzky CM, Lavelle MT, Garcia RA. Best Cases from the AFIP: Cystic pheochromocytoma. Radiographics 2002;22:935-40. |
|3.||Dluhy RG. Pheochromocytoma -- death of an Axiom. N Engl J Med 2002;346:1486-8. |
|4.||Madani R, Al-Hashmi M, Bliss R, Lennard TW. Ectopic pheochromocytoma: does the rule of tens apply?. World J Surg 2007;31:849-54. |
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]
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