Extra-adrenal pheochromocytoma at the organ of Zuckerkandl: a case report and literature review

Gill, Tarana; Adler, Kalie; Schrader, Alicia; Desai, Keyur; Wermers, Joshua D; Beteselassie, Nebiyu

doi:10.1016/j.radcr.2016.12.009

Cited by 9 publications

(11 citation statements)

References 7 publications

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“…11 Heterogeneity of the parenchyma is also a common finding in retroperitoneal paragangliomas in people as cystic changes, necrosis, hemorrhage, and mineralization are frequent. 1,23,25 The included MRI showed an amorphous heterogeneous mass. Paragangliomas often display a "salt-and-pepper" appearance in people, as flow voids from vascularity create punctate areas of low signal intensity and hemorrhage causes areas of hyperintense signal.…”

Section: Discussionmentioning

confidence: 98%

Imaging features of retroperitoneal extra‐adrenal paragangliomas in 10 dogs

Gombert

Diana

Hecht

et al. 2022

Vet Radiology Ultrasound

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Retroperitoneal paragangliomas are rare tumors of the neuroendocrine system. Only a few canine case reports are available with rare descriptions of their imaging features.The objectives of this multi-center, retrospective case series study were to describe the diagnostic imaging features of confirmed retroperitoneal paragangliomas and specify their location. Medical records and imaging studies of 10 affected dogs with cytological or histopathologic results concordant with retroperitoneal paragangliomas were evaluated. Dogs had a median age of 9 years. Four of them had clinical signs and laboratory reports compatible with excessive production of catecholamines. Six ultrasound, four CT, four radiographic, and one MRI studies were included. The paragangliomas did not have a specific location along the aorta. They were of various sizes (median 33 mm, range: 9-85 mm of length). Masses had heterogeneous parenchyma in six of 10 dogs, regardless of the imaging modality. Strong contrast enhancement was found in all CT studies. Encircling of at least one vessel was detected in six of 10 masses, clear invasion of a vessel was identified in one of 10 masses. In five of 10 cases, the masses were initially misconstrued as lymph nodes by the on-site radiologist. Retroperitoneal paragangliomas appear along the abdominal aorta, often presenting heterogeneous parenchyma, possibly affecting the local vasculature, and displaying strong contrast enhancement on CT. Clinical signs can be secondary to mass effects or excessive catecholamine production. Underdiagnosis and misdiagnosis of this tumor are suspected as they can be silent, of small size, or confused with other structures.

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Section: Discussionmentioning

confidence: 98%

Imaging features of retroperitoneal extra‐adrenal paragangliomas in 10 dogs

Gombert

Diana

Hecht

et al. 2022

Vet Radiology Ultrasound

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“…PGLs are rare catecholamine-secreting neuroendocrine tumors that arise from the extra-adrenal paraganglia. Most PGLs occur in the subdiaphragmatic region, most commonly within the organ of Zuckerkandl [8]. The clinical presentation of pheochromocytomas/PGLs can vary remarkably, from dramatic symptoms and signs to minimal or no symptoms whatsoever [9].…”

Section: Discussionmentioning

confidence: 99%

Laparoscopic resection of a large clinically silent paraganglioma at the organ of Zuckerkandl: a rare case report and review of the literature

Ren¹,

Shang²,

Ren³

et al. 2020

BMC Urol

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Background Large paraganglioma of the Zuckerkandl organ (POZ) is extremely rare. The patient can occasionally be paucisymptomatic, further obscuring the diagnosis and carrying high mortality. Recommended treatment for large paraganglioma (PGL) is open surgical removal. We report a case of successful laparoscopic resection of a large POZ with normal blood pressure in a 45-year-old man. Case presentation A 45-year-old man was hospitalized because of hyperglycemia. Computed tomography of the abdomen and the serum and urinary catecholamine levels confirmed the diagnosis of large POZ. But his blood pressure was normal and he underwent laparoscopic tumor excision successfully. During 6 months follow-up after laparoscopy, serum and urinary catecholamines were normal but glycaemia remained high level. DNA analysis of the succinate dehydrogenase complex subunits B (SDHB) and SDHD revealed no mutation. Conclusions POZ is an unusual mass and preoperative diagnosis can be difficult in clinically silent cases. PGL cannot be excluded in patients with normal blood pressure. Even a large POZ can be excised laparoscopically by following proper techniques.

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“…1,2 Most EAPs occur below the diaphragm, most commonly at the organ of Zuckerkandl, the collection of para-aortic chromaffin tissue just below the origin of the inferior mesenteric artery. 3,4…”

Section: Discussionmentioning

confidence: 99%

Open abdominal aortic aneurysm repair with incidental finding of an extra-adrenal pheochromocytoma of the organ of Zuckerkandl

Gonzalez

Singh

2020

Vascular

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Objectives Pheochromocytomas are rare catecholamine-secreting neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla or extra-adrenal paraganglia. The most common location of these tumors is within the adrenal medulla. Extra-adrenal pheochromocytomas (EAPs) may occur in any portion of the paraganglion system. The most common location of EAPs is at the organ of Zuckerkandl, which is a collection of chromaffin cells near the origin of the inferior mesenteric artery. Methods We present a case of an EAP of the organ of Zuckerkandl incidentally discovered and resected during urgent open repair of a symptomatic 6.7-cm juxtarenal abdominal aortic aneurysm (AAA). Results The patient underwent successful open surgical repair of a juxtarenal AAA and resection of the pheochromocytoma. Conclusions Concomitant pheochromocytomas and abdominal aortic aneurysms are rare, with a small number described in the literature. We describe the case of a simultaneous EAP of the organ of Zuckerkandl and AAA repair. This case demonstrates that these lesions can be safely resected in the same setting as AAA repair.

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Extra-adrenal pheochromocytoma at the organ of Zuckerkandl: a case report and literature review

Cited by 9 publications

References 7 publications

Imaging features of retroperitoneal extra‐adrenal paragangliomas in 10 dogs

Imaging features of retroperitoneal extra‐adrenal paragangliomas in 10 dogs

Laparoscopic resection of a large clinically silent paraganglioma at the organ of Zuckerkandl: a rare case report and review of the literature

Open abdominal aortic aneurysm repair with incidental finding of an extra-adrenal pheochromocytoma of the organ of Zuckerkandl

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