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
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
Peard L et al: Pediatric pheochromocytoma: current status of diagnostic imaging and treatment procedures. Curr Opin Urol. 29(5):493-9, 2019
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
Sargar KM et al: Imaging of nonmalignant adrenal lesions in children. Radiographics. 37(6):1648-64, 2017
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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
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
Peard L et al: Pediatric pheochromocytoma: current status of diagnostic imaging and treatment procedures. Curr Opin Urol. 29(5):493-9, 2019
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
Sargar KM et al: Imaging of nonmalignant adrenal lesions in children. Radiographics. 37(6):1648-64, 2017
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