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Pediatrics
Diagnosis
Genitourinary
Adrenal Abnormalities
Pheochromocytoma
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

            • Hereditary predisposition in up to 40%
              • Mutations in succinate dehydrogenase (SDH), von Hippel-Lindau syndrome, multiple endocrine neoplasia type 2, & neurofibromatosis type 1
            • Identifiable germline mutation in 70-80% of patients

          IMAGING

          • General Features

            • Ultrasonographic Findings

              • CT Findings

                • MR Findings

                  • Nuclear Medicine Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        CLINICAL ISSUES

                        • Presentation

                          • Demographics

                            • Natural History & Prognosis

                              • Treatment

                                Selected References

                                1. Jochmanova I et al: Clinical characteristics and outcomes of SDHB-related pheochromocytoma and paraganglioma in children and adolescents. J Cancer Res Clin Oncol. 146(4):1051-63, 2020
                                2. Peard L et al: Pediatric pheochromocytoma: current status of diagnostic imaging and treatment procedures. Curr Opin Urol. 29(5):493-9, 2019
                                3. Jha A et al: Superiority of 68Ga-DOTATATE over 18F-FDG and anatomic imaging in the detection of succinate dehydrogenase mutation (SDHx )-related pheochromocytoma and paraganglioma in the pediatric population. Eur J Nucl Med Mol Imaging. 45(5):787-97, 2018
                                4. Sargar KM et al: Imaging of nonmalignant adrenal lesions in children. Radiographics. 37(6):1648-64, 2017
                                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

                                          • Hereditary predisposition in up to 40%
                                            • Mutations in succinate dehydrogenase (SDH), von Hippel-Lindau syndrome, multiple endocrine neoplasia type 2, & neurofibromatosis type 1
                                          • Identifiable germline mutation in 70-80% of patients

                                        IMAGING

                                        • General Features

                                          • Ultrasonographic Findings

                                            • CT Findings

                                              • MR Findings

                                                • Nuclear Medicine Findings

                                                  • Imaging Recommendations

                                                    DIFFERENTIAL DIAGNOSIS

                                                      CLINICAL ISSUES

                                                      • Presentation

                                                        • Demographics

                                                          • Natural History & Prognosis

                                                            • Treatment

                                                              Selected References

                                                              1. Jochmanova I et al: Clinical characteristics and outcomes of SDHB-related pheochromocytoma and paraganglioma in children and adolescents. J Cancer Res Clin Oncol. 146(4):1051-63, 2020
                                                              2. Peard L et al: Pediatric pheochromocytoma: current status of diagnostic imaging and treatment procedures. Curr Opin Urol. 29(5):493-9, 2019
                                                              3. Jha A et al: Superiority of 68Ga-DOTATATE over 18F-FDG and anatomic imaging in the detection of succinate dehydrogenase mutation (SDHx )-related pheochromocytoma and paraganglioma in the pediatric population. Eur J Nucl Med Mol Imaging. 45(5):787-97, 2018
                                                              4. Sargar KM et al: Imaging of nonmalignant adrenal lesions in children. Radiographics. 37(6):1648-64, 2017