Abstract

Extra-adrenal paraganglioma of the retroperitoneum are rare neuroendocrine tumors arising from specialized neural crest cells, intimately associated with sympathetic nervous tissue and distributed along the aorta. Here we present a case of multiple extra-adrenal retroperitoneal paraganglioma in a 16-year-old boy. The clinical presentation, imaging characteristics, and surgical pathologic findings were reported. A comprehensive review of the medical literature concerning multiple extra-adrenal paraganglioma in the pediatric and adult population is also discussed.

Highlights

  • Extra-adrenal retroperitoneal paraganglioma are rare neuroendocrine tumors arising from specialized neural crest cells, intimately associated with sympathetic nervous tissue and distributed along the aorta [1]

  • The general designation “paraganglioma” applies to tumors arising from paraganglia regardless of location

  • The only exception is the paraganglioma of the adrenal medulla, which is universally known as pheochromocytoma

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Summary

Open Access

Bi-Focal Retroperitoneal Paraganglioma in a Young Patient: A Case Report and Review of the Literature.

Introduction
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