CASE 2284 Published on 02.03.2004

Extraadrenal pheochromocytoma based in organ of Zuckerkandl.

Section

Cardiovascular

Case Type

Clinical Cases

Authors

Maudgil DD, Bell J, Hamilton G, Platts AD, Watkinson AF.

Patient

34 years, female

Categories

No Area of Interest ; Imaging Technique CT, Nuclear medicine conventional

No Procedure ; No Special Focus ;

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Clinical History

1 year history of arterial hypertension

Imaging Findings

The patient presented with a 1 year history of arterial hypertension, headaches and migraine. The patient underwent CT, MIBG, venography and aortography.

Discussion

Pheochromocytomas may present with vague symptoms due to excess catecholamine production: the majority present with paroxysmal (47%) or sustained (37%) hypertension, although other presentations include nausea, headaches or flushing. Urinary VMA (vanillyl mandelic acid) excretion is elevated in 54%. They may occur as part of a multiple endocrine neoplasia syndrome, or in association with a neuroectodermal disorder such as tuberous sclerosis or Von Hippel-Lindau syndrome.
Pheochromoctyomas have been called the “10% tumour” since:
- 10% are located outside the adrenal glands (particularly in the para-aortic sympathetic chain or the organ of Zuckerkandl);
- 10% are malignant (although 40% of extra-adrenal tumours are);
- 10% are familial;
- 10% are bilateral or multiple.

Treatment is by surgical resection after pharmacological blockade. This patient was a Jehovah’s Witness and completely refused blood transfusion. Therefore pre-operative embolisation (with pharmacological blockade) was performed with PVA particles. This was successfully performed and allowed complete surgical resection with minimal blood loss at operation.

Differential Diagnosis List

Extraadrenal pheochromocytoma based in organ of Zuckerkandl.

Final Diagnosis

Extraadrenal pheochromocytoma based in organ of Zuckerkandl.

References

[1] Dahnert W.<br> Radiology review manual 4th Ed. <br>Williams and Wilkins, Baltimore.

[2] Ali-el-Dein B, El-Sobky E, El-Baz M, Shaaban AA.
Abdominal and pelvic extra-adrenal paraganglioma: a review of literature and a report on 7 cases.
In Vivo 2002 Jul-Aug;16(4):249-54. (PMID: 12224134)

[3] Herrera LO, Hossain ZM, Rafal HS, Frelick RW, Ashley PF, Lopez GE.
Malignant pheochromocytoma (paraganglioma) of the organ of Zuckerkandl: a study of two cases.
J Surg Oncol 1980;14(2):133-45. (PMID: 6446627)

[4] Baraka A, Siddik-Sayyid S, Jalbout M, Yaacoub C.
Variable hemodynamic fluctuations during resection of multicentric extraadrenal pheochromocytomas.
Can J Anaesth 2002 Aug;49(7):682-6. (PMID: 6446627)

[5] Ichikawa T, Fujimoto H, Murakami K, Ishikawa N, Itami J, Arimizu N.
Extraadrenal pheochromocytoma: dynamic demonstration at 0.5 tesla.
Clin Imaging 1992 Jul-Sep;16(3):187-9. (PMID: 1323372)

Case information

URL:	https://www.eurorad.org/case/2284
DOI:	10.1594/EURORAD/CASE.2284
ISSN:	1563-4086

Figure 1

CT scan of the abdomen

CT scan demonstrates a 5cm soft tissue retroperitoneal mass in the region of the lower abdomen. The mass demonstrates peripheral vascular enhancement with intravenous contrast in the arterial phase.

Figure 2

MIBG scan of the abdomen

MIBG scan demonstrates uptake in an area corresponding to the mass seen on CT.

Figure 3

Venography and aortography

Venography via a left femoral vein puncture demonstrates extrinsic compression of the left common iliac vein by a rounded mass.

Aortography via a right common femoral artery approach demonstrates a complex vascular mass inferior to the origin of the inferior mesenteric artery, which has a prominent vascular blush and takes a feeding supply from several vessels in this region including the superior mesenteric artery.