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Pheochromocytoma
Alexander J. Towbin, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Definitions

            • Paraganglioma arising from catecholamine-producing chromaffin cells of adrenal medulla
            • Extraadrenal paraganglioma arises from sympathetic or parasympathetic paraganglia
          • Associated Syndromes

            • von Hippel-Lindau, multiple endocrine neoplasia syndrome type 2, neurofibromatosis type 1
            • Carney triad: Nonfamilial paraganglioma, gastrointestinal stromal tumor, & pulmonary chondroma
            • Identifiable germline mutation in 59% of patients < 18 years of age & 70% of patients < 10 years of age

          IMAGING

          • General Features

            • CT Findings

              • MR Findings

                • Ultrasonographic Findings

                  • Nuclear Medicine Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        CLINICAL ISSUES

                        • Presentation

                          • Demographics

                            • Natural History & Prognosis

                              • Treatment

                                Selected References

                                1. Bittman ME et al: Focal adrenal lesions in pediatric patients. AJR Am J Roentgenol. 200(6):W542-56, 2013
                                2. Balassy C et al: Adrenal masses in children. Radiol Clin North Am. 49(4):711-27, vi, 2011
                                3. Edmonds S et al: Pheochromocytoma. Pediatr Rev. 32(7):308-10, 2011
                                4. Waguespack SG et al: A current review of the etiology, diagnosis, and treatment of pediatric pheochromocytoma and paraganglioma. J Clin Endocrinol Metab. 95(5):2023-37, 2010
                                5. Pham TH et al: Pheochromocytoma and paraganglioma in children: a review of medical and surgical management at a tertiary care center. Pediatrics. 118(3):1109-17, 2006
                                Related Anatomy
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                                Related Differential Diagnoses
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                                References
                                Tables

                                Tables

                                KEY FACTS

                                • Terminology

                                  • Imaging

                                    • Top Differential Diagnoses

                                      • Clinical Issues

                                        TERMINOLOGY

                                        • Definitions

                                          • Paraganglioma arising from catecholamine-producing chromaffin cells of adrenal medulla
                                          • Extraadrenal paraganglioma arises from sympathetic or parasympathetic paraganglia
                                        • Associated Syndromes

                                          • von Hippel-Lindau, multiple endocrine neoplasia syndrome type 2, neurofibromatosis type 1
                                          • Carney triad: Nonfamilial paraganglioma, gastrointestinal stromal tumor, & pulmonary chondroma
                                          • Identifiable germline mutation in 59% of patients < 18 years of age & 70% of patients < 10 years of age

                                        IMAGING

                                        • General Features

                                          • CT Findings

                                            • MR Findings

                                              • Ultrasonographic Findings

                                                • Nuclear Medicine Findings

                                                  • Imaging Recommendations

                                                    DIFFERENTIAL DIAGNOSIS

                                                      CLINICAL ISSUES

                                                      • Presentation

                                                        • Demographics

                                                          • Natural History & Prognosis

                                                            • Treatment

                                                              Selected References

                                                              1. Bittman ME et al: Focal adrenal lesions in pediatric patients. AJR Am J Roentgenol. 200(6):W542-56, 2013
                                                              2. Balassy C et al: Adrenal masses in children. Radiol Clin North Am. 49(4):711-27, vi, 2011
                                                              3. Edmonds S et al: Pheochromocytoma. Pediatr Rev. 32(7):308-10, 2011
                                                              4. Waguespack SG et al: A current review of the etiology, diagnosis, and treatment of pediatric pheochromocytoma and paraganglioma. J Clin Endocrinol Metab. 95(5):2023-37, 2010
                                                              5. Pham TH et al: Pheochromocytoma and paraganglioma in children: a review of medical and surgical management at a tertiary care center. Pediatrics. 118(3):1109-17, 2006