DOI: 10.363247/sjmcr.2018.v06i10.025

Pages: 834-837

Retroperitoneal paragangliomas are rare tumors. They are defined as extra-adrenal chromaffin tumors and represent approximately 1 / 5th of chromaffin tumors. They are often asymptomatic and can reach important dimensions. We report the case of a 7-year-old boy operated on for a giant retroperitoneal tumor whose pathological examination concluded that he had a paraganglioma. The malignant forms, more frequent than the benign forms, present a locoregional invasion and metastasize late. Management of paragangliomas should be multidisciplinary but only surgical treatment is curative. On the other hand, there is no consensus on the usefulness of complementary therapies, which may nonetheless be supportive as a symptom. Paragangliomas are genetic in 25% of cases. A genetic survey must be systematically proposed.