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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
- Sympathetic: Found along sympathetic chain from neck to bladder
- 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
- Paraganglioma
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
- Corssmit EPM et al: Malignant pheochromocytoma and paraganglioma: management options. Curr Opin Oncol. 32(1):20-6, 2020
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
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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
- Sympathetic: Found along sympathetic chain from neck to bladder
- 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
- Paraganglioma
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
- Corssmit EPM et al: Malignant pheochromocytoma and paraganglioma: management options. Curr Opin Oncol. 32(1):20-6, 2020
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
