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Pheochromocytoma and Paraganglioma
Paige Bennett, MD
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KEY FACTS

  • Terminology

    • Pathology

      • Clinical Issues

        • Diagnostic Checklist

          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
                • Sympathetic: Found along sympathetic chain from neck to bladder
                  • 75% have origin in abdomen
                  • Organ of Zuckerkandl near aortic bifurcation most common site
                  • High rate (~ 85%) of excess catecholamine secretion
                • Parasympathetic: Found around carotid body, along course of vagus and glossopharyngeal nerves
                  • Majority arise in head and neck
                  • Head and neck paragangliomas are highly associated (~ 50%) with genetic syndromes
                  • Do not secrete catecholamines
              • Majority sporadic
                • However, 25% are associated with genetic syndromes
            • Pheochromocytoma is intraadrenal sympathetic paraganglioma
              • Tumor arising from chromaffin cells of adrenal medulla
              • Secretes epinephrine and norepinephrine
              • 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

          • Ga-68 Dotatate PET/CT Findings

            • F-18 FDG PET/CT Findings

              • I-123/I-131 MIBG and In-111 Octreotide

                • CECT Findings

                  DIFFERENTIAL DIAGNOSIS

                    PATHOLOGY

                    • General Features

                      • Staging, Grading, & Classification

                        CLINICAL ISSUES

                        • Presentation

                          • Natural History & Prognosis

                            DIAGNOSTIC CHECKLIST

                            • Consider

                              Selected References

                              1. Corssmit EPM et al: Malignant pheochromocytoma and paraganglioma: management options. Curr Opin Oncol. 32(1):20-6, 2020
                              2. Parra LA et al: Multifocal urinary bladder paragangliomas with negative 68Ga-DOTATATE uptake and positive 123I-MIBG uptake. Clin Nucl Med. 45(3):e156-7, 2020
                              3. Shahrokhi P et al: 68Ga-DOTATATE PET/CT compared with 131I-MIBG SPECT/CT in the evaluation of neural crest tumors. Asia Ocean J Nucl Med Biol. 8(1):8-17, 2020
                              4. Skoura E et al: The impact of Ga-68 DOTATATE PET/CT imaging on management of patients with paragangliomas. Nucl Med Commun. 41(2):169-74, 2020
                              5. 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
                              6. 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
                              7. 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
                              8. 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
                              9. 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

                                    • Diagnostic Checklist

                                      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
                                            • Sympathetic: Found along sympathetic chain from neck to bladder
                                              • 75% have origin in abdomen
                                              • Organ of Zuckerkandl near aortic bifurcation most common site
                                              • High rate (~ 85%) of excess catecholamine secretion
                                            • Parasympathetic: Found around carotid body, along course of vagus and glossopharyngeal nerves
                                              • Majority arise in head and neck
                                              • Head and neck paragangliomas are highly associated (~ 50%) with genetic syndromes
                                              • Do not secrete catecholamines
                                          • Majority sporadic
                                            • However, 25% are associated with genetic syndromes
                                        • Pheochromocytoma is intraadrenal sympathetic paraganglioma
                                          • Tumor arising from chromaffin cells of adrenal medulla
                                          • Secretes epinephrine and norepinephrine
                                          • 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

                                      • Ga-68 Dotatate PET/CT Findings

                                        • F-18 FDG PET/CT Findings

                                          • I-123/I-131 MIBG and In-111 Octreotide

                                            • CECT Findings

                                              DIFFERENTIAL DIAGNOSIS

                                                PATHOLOGY

                                                • General Features

                                                  • Staging, Grading, & Classification

                                                    CLINICAL ISSUES

                                                    • Presentation

                                                      • Natural History & Prognosis

                                                        DIAGNOSTIC CHECKLIST

                                                        • Consider

                                                          Selected References

                                                          1. Corssmit EPM et al: Malignant pheochromocytoma and paraganglioma: management options. Curr Opin Oncol. 32(1):20-6, 2020
                                                          2. Parra LA et al: Multifocal urinary bladder paragangliomas with negative 68Ga-DOTATATE uptake and positive 123I-MIBG uptake. Clin Nucl Med. 45(3):e156-7, 2020
                                                          3. Shahrokhi P et al: 68Ga-DOTATATE PET/CT compared with 131I-MIBG SPECT/CT in the evaluation of neural crest tumors. Asia Ocean J Nucl Med Biol. 8(1):8-17, 2020
                                                          4. Skoura E et al: The impact of Ga-68 DOTATATE PET/CT imaging on management of patients with paragangliomas. Nucl Med Commun. 41(2):169-74, 2020
                                                          5. 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
                                                          6. 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
                                                          7. 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
                                                          8. 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
                                                          9. 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