Abdomen / Pelvis for Wednesday, April 9th, 2025

History

Previously healthy 12-year-old male presenting with chest, pain, diaphoresis, headache, and hypertension.

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Question

What is the most likely diagnosis?

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Correct answer

Paraganglioma

Discussion

Lab evaluation demonstrated normal urine VMA/HMA with markedly elevated urine norepinephrine levels. 

The patient received blood pressure control with initially a calcium channel blocker. Preoperatively, alpha blockade was achieved then beta blockade for tachycardia. The resection was complicated by hypertension with tumor manipulation, anti-hypertensive infusion and ligation of the feeding vessels controlled the hypertension. Inotropic support and fluid boluses were given for subsequent hypotension.

Pathology report confirmed paraganglioma.  Paragangliomas are neuroendocrine tumors that arise from paraganglia and are most commonly found in several specific locations in the head and neck, the mediastinum and in the retroperitoneum around the aorta. A paraganglioma of the adrenal gland is more commonly known as a pheochromocytoma. This case of abdominal paraganglioma could also be called an "extra-adrenal pheochromocytoma."

They can occur at any age without sex bias but are most common in middle age. Paragangliomas that secrete excess catecholamines such as norepinephrine are more common in the mediastinum and the retroperitoneum. Common clinical symptoms include hypertension, headache, sweating and palpitations. 

On contrasted CT, abdominal paragangliomas usually appear as enhancing masses frequently in the infrarenal region. They may contain calcification and focal areas of high or low attenuation. On MRI, they are typically T2 hyperintense and T1 hypo or iso intense in comparison with liver. MIBG scintigraphy is very sensitive for detection of paragangliomas and can also provide information on function. In this case, an additional smaller tumor was also found at the iliac bifurcation that was also excised and confirmed as paraganglioma. 

 Catecholamine induced hypertension is a potentially life-threatening complication of paragangliomas and pheochromocytomas, up to 80 percent of patients develop a hypertensive crisis and 23-30% experience hypotension during surgery/treatment including the patient in this case.

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References

• Lee, K. Y., Oh, Y. W., Noh, H. J., Lee, Y. J., Yong, H. S., Kang, E. Y., ... & Lee, N. J. (2006). Extraadrenal paragangliomas of the body: imaging features. American Journal of Roentgenology, 187(2), 492-504. Nazari, M. A., Hasan, R., Haigney, M., Maghsoudi, A., Lenders, J. W., Carey, R. M., & Pacak, K. (2023). Catecholamine-induced hypertensive crises: current insights and management. The Lancet Diabetes & Endocrinology.