Organ of Zuckerkandl explained

The organ of Zuckerkandl is a chromaffin body derived from the neural crest located at the bifurcation of the aorta or at the origin of the inferior mesenteric artery. It can be the source of a paraganglioma.^[1]

The term para-aortic body is also sometimes used to describe it, as it usually arises near the abdominal aorta, but this term can be the source of confusion, because the term "corpora paraaortica" is also used to describe the aortic body, which arises near the thoracic aorta. This diffused group of neuroendocrine sympathetic fibres was first described by Emil Zuckerkandl, a professor of anatomy at the University of Vienna, in 1901.

Some sources equate the "aortic bodies" and "paraaortic bodies", while other sources explicitly distinguish between the two. When a distinction is made, the "aortic bodies" are chemoreceptors which regulate circulation, while the "paraaortic bodies" are the chromaffin cells which manufacture catecholamines.

Organs of Zuckerkandl (O of Z) harbor the potential for deadly paragangliomas. Paragangliomas are one of the surgical causes of hypertension. Major treatises of medicine offer very little information on this topic.^[2]

Function

Its physiological role is thought to be of the greatest importance during the early gestational period as a homeostatic regulator of blood pressure, secreting catecholamines into the fetal circulation.

The organ itself regresses in the late third trimester and following birth to form the aorticosympathetic group of the adult paraganglia.^[3]

Clinical significance

Pathology

It can be the source of paraganglioma.^[4]

The organ of Zuckerkandl is of pathological significance in the adult as a common extra-adrenal site of pheochromocytoma though the most common extra-adrenal site is in the superior para-aortic region between the diaphragm and lower renal poles.^[5]

Extra-adrenal tumors account for around 25% of all cases of pheochromocytoma.^[6] Resection has been described.^[7]

In addition to its endocrine effects, it can also cause obstructive pathology.^[8]

Notes and References

1. 10.4158/ep.13.5.493 . 17872352 . Malignant Pheochromocytoma of the Organ of Zuckerkandl Requiring Aortic and Vena Caval Reconstruction . Endocrine Practice . 13 . 5 . 493–7 . 2007 . Dossett . Lesly . Rudzinski . Erin . Blevins . Lewis . Chambers . Eugene .
2. Subramanian . Anuradha . Maker . Vijay K. . 2006-08-01 . Organs of Zuckerkandl: their surgical significance and a review of a century of literature . The American Journal of Surgery . en . 192 . 2 . 224–234 . 10.1016/j.amjsurg.2006.02.018 . 16860635 . 0002-9610.
3. Schober. Andreas. Parlato. Rosanna. Huber. Katrin. Kinscherf. Ralf. Hartleben. Björn. Huber. Tobias B.. Schütz. Günther. Unsicker. Klaus. Cell Loss and Autophagy in the Extra-Adrenal Chromaffin Organ of Zuckerkandl are Regulated by Glucocorticoid Signalling. Journal of Neuroendocrinology. 25. 1. 2013. 34–47. 0953-8194. 10.1111/j.1365-2826.2012.02367.x. 23078542. 3564403.
4. 10.1148/rg.235035022 . 12975514 . Best Cases from the AFIP . RadioGraphics . 23 . 5 . 1279–86 . 2003 . Saurborn . Daniel P. . Kruskal . Jonathan B. . Stillman . Isaac E. . Parangi . Sareh .
5. 1729490 . 1992 . Whalen . R. K. . Extra-adrenal pheochromocytoma . The Journal of Urology . 147 . 1 . 1–10 . Althausen . A. F. . Daniels . G. H. . 10.1016/s0022-5347(17)37119-7 .
6. 10.1056/NEJM200205093461910 . 12000820 . Detection and Management of Small Aortic Aneurysms . New England Journal of Medicine . 346 . 19 . 1484–6 . 2002 . Thompson . Robert W. .
7. 10.1016/j.jpedsurg.2007.06.025 . 17923216 . Laparoscopic resection of extra-adrenal pheochromocytoma—Case report and review of the literature in pediatric patients . Journal of Pediatric Surgery . 42 . 10 . 1780–4 . 2007 . Kravarusic . Dragan . Pinto-Rojas . Alfredo . Al-Assiri . Ali . Sigalet . David .
8. 10.1016/j.jamcollsurg.2005.02.031 . 16125084 . Massive Organ of Zuckerkandl Inducing Small Bowel Obstruction . Journal of the American College of Surgeons . 201 . 3 . 480–1 . 2005 . Blecha . Matthew . Galanopolous . Christos . Dharkar . Dilip . Salti . George .