Presentation
Refractory hypertension. Cause?
Patient Data
In the retroperitoneum lying between the abdominal aorta and IVC, just inferior to the left renal vein and superior mesenteric artery, is a mildly lobulated, well-defined mass. The mass demonstrates vivid rim enhancement with an irregular central hypodense/hypoenhancing component. The right renal artery is compressed by the mass, and the right kidney is atrophic. The adrenals are unremarkable.
Right direct inguinal fat-containing hernia is noted.
Differential diagnoses for the retroperitoneal mass include metastasis and paraganglioma (particularly in view of the clinical history of hypertension). The appearance is not typical for lymphoma.
Case Discussion
CLINICAL NOTES: Organ of Zuckerkandl paraganglioma. Initially presented with hypertension. Lesion found between aorta + IVC. Increased urinary catacholamines + increased plasma metanephrine ?Genetic cause -> ? succinate dehydrogenase (SDH) subunit gene mutation.
MACROSCOPIC DESCRIPTION: "Organ of Zuckerkandl paraganglioma short superior, long left": A soft tan mass weighing 40g and measuring 60x40x35mm.
MICROSCOPIC DESCRIPTION: The mass is encapsulated and surrounded by predominantly fatty soft tissue. It is composed of large polygonal epithelioid cells arranged in nests in a sparse fibrovascular stroma with scattered areas of fibrosis. The cells have clear or granular eosinophilic cytoplasm and rounded or oval nuclei with finely granular chromatin and small nucleoli. Scattered cells have large or bizarre nuclei. No mitoses are seen and there is no necrosis. No lymphovascular invasion is found. Excision is complete. Less than 1% of the cells are Ki67+.
Further SDH study reports: "Immunohistochemistry is as follows: SDHB - positive. SDHA - positive. Fumarate hydratase - positive. This is a normal pattern of staining which makes SDHX mutation unlikely."
DIAGNOSIS:
Lesion organ of Zuckerkandl: Paraganglioma with no adverse histological features.
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