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Paraganglioma and Pheochromocytoma
Paige Bennett, MD
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KEY FACTS

  • Terminology

    • Pathology

      • Clinical Issues

        TERMINOLOGY

        • Definitions

          • Paraganglioma
            • Neuroendocrine tumor arising from chromoceptor/chromaffin cells of paraganglion
            • Intraabdominal (85%), intrathoracic (12%), head and neck (3%)
            • Arises from sympathetic and parasympathetic ganglion
              • May not secrete catecholamines
              • Sympathetic: Organ of Zuckerkandl near aortic bifurcation, along sympathetic chain from neck to bladder
              • Parasympathetic: Carotid body, along course of vagus and glossopharyngeal nerves; do not secrete catecholamines
          • Pheochromocytoma
            • Tumor arising from chromaffin cells of adrenal medulla
            • Secretes epinephrine and norepinephrine
            • Intraadrenal sympathetic paraganglioma
            • Risk of malignancy > 10%
            • ~ 30% genetically based
          • Symptoms of catecholamine-secreting paraganglioma and pheochromocytoma
            • Episodic severe hypertension, headache, palpitations, diaphoresis
            • 1/2 of patients have persistent hypertension that is labile/resistant to therapy
            • ~ 30% of patients have normal blood pressure

        IMAGING

        • F-18 FDG PET/CT Findings

          • Ga-68 DOTA-Peptides PET/CT Findings

            • CECT Findings

              DIFFERENTIAL DIAGNOSIS

                PATHOLOGY

                • General Features

                  • Staging, Grading, & Classification

                    CLINICAL ISSUES

                    • Presentation

                      • Natural History & Prognosis

                        DIAGNOSTIC CHECKLIST

                        • Consider

                          Selected References

                          1. Archier A et al: Prospective comparison of (68)Ga-DOTATATE and (18)F-FDOPA PET/CT in patients with various pheochromocytomas and paragangliomas with emphasis on sporadic cases. Eur J Nucl Med Mol Imaging. 43(7):1248-57, 2016
                          2. Chang CA et al: (68)Ga-DOTATATE and (18)F-FDG PET/CT in paraganglioma and pheochromocytoma: utility, patterns and heterogeneity. Cancer Imaging. 16(1):22, 2016
                          3. Janssen I et al: PET/CT comparing (68)Ga-DOTATATE and other radiopharmaceuticals and in comparison with CT/MRI for the localization of sporadic metastatic pheochromocytoma and paraganglioma. Eur J Nucl Med Mol Imaging. 43(10):1784-91, 2016
                          4. Timmers HJ et al: Staging and functional characterization of pheochromocytoma and paraganglioma by 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography. J Natl Cancer Inst. 104(9):700-8, 2012
                          5. Timmers HJ et al: Comparison of 18F-fluoro-L-DOPA, 18F-fluoro-deoxyglucose, and 18F-fluorodopamine PET and 123I-MIBG scintigraphy in the localization of pheochromocytoma and paraganglioma. J Clin Endocrinol Metab. 94(12):4757-67, 2009
                          Related Anatomy
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                          Related Differential Diagnoses
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                          References
                          Tables

                          Tables

                          KEY FACTS

                          • Terminology

                            • Pathology

                              • Clinical Issues

                                TERMINOLOGY

                                • Definitions

                                  • Paraganglioma
                                    • Neuroendocrine tumor arising from chromoceptor/chromaffin cells of paraganglion
                                    • Intraabdominal (85%), intrathoracic (12%), head and neck (3%)
                                    • Arises from sympathetic and parasympathetic ganglion
                                      • May not secrete catecholamines
                                      • Sympathetic: Organ of Zuckerkandl near aortic bifurcation, along sympathetic chain from neck to bladder
                                      • Parasympathetic: Carotid body, along course of vagus and glossopharyngeal nerves; do not secrete catecholamines
                                  • Pheochromocytoma
                                    • Tumor arising from chromaffin cells of adrenal medulla
                                    • Secretes epinephrine and norepinephrine
                                    • Intraadrenal sympathetic paraganglioma
                                    • Risk of malignancy > 10%
                                    • ~ 30% genetically based
                                  • Symptoms of catecholamine-secreting paraganglioma and pheochromocytoma
                                    • Episodic severe hypertension, headache, palpitations, diaphoresis
                                    • 1/2 of patients have persistent hypertension that is labile/resistant to therapy
                                    • ~ 30% of patients have normal blood pressure

                                IMAGING

                                • F-18 FDG PET/CT Findings

                                  • Ga-68 DOTA-Peptides PET/CT Findings

                                    • CECT Findings

                                      DIFFERENTIAL DIAGNOSIS

                                        PATHOLOGY

                                        • General Features

                                          • Staging, Grading, & Classification

                                            CLINICAL ISSUES

                                            • Presentation

                                              • Natural History & Prognosis

                                                DIAGNOSTIC CHECKLIST

                                                • Consider

                                                  Selected References

                                                  1. Archier A et al: Prospective comparison of (68)Ga-DOTATATE and (18)F-FDOPA PET/CT in patients with various pheochromocytomas and paragangliomas with emphasis on sporadic cases. Eur J Nucl Med Mol Imaging. 43(7):1248-57, 2016
                                                  2. Chang CA et al: (68)Ga-DOTATATE and (18)F-FDG PET/CT in paraganglioma and pheochromocytoma: utility, patterns and heterogeneity. Cancer Imaging. 16(1):22, 2016
                                                  3. Janssen I et al: PET/CT comparing (68)Ga-DOTATATE and other radiopharmaceuticals and in comparison with CT/MRI for the localization of sporadic metastatic pheochromocytoma and paraganglioma. Eur J Nucl Med Mol Imaging. 43(10):1784-91, 2016
                                                  4. Timmers HJ et al: Staging and functional characterization of pheochromocytoma and paraganglioma by 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography. J Natl Cancer Inst. 104(9):700-8, 2012
                                                  5. Timmers HJ et al: Comparison of 18F-fluoro-L-DOPA, 18F-fluoro-deoxyglucose, and 18F-fluorodopamine PET and 123I-MIBG scintigraphy in the localization of pheochromocytoma and paraganglioma. J Clin Endocrinol Metab. 94(12):4757-67, 2009