Imaging Pearls ❯ Pancreas ❯ Paragangliomas
|
-- OR -- |
|
- “Paragangliomas are tumors arising from the extra-adrenal paraganglia, which are nests of specialized neural crest cells dispersed along the sympathetic ganglia and plexuses throughout the body, including the chemoreceptors (carotid and aortic bodies), vagal body, and thoracic, intra-abdominal, and retroperitoneal ganglia. Retroperitoneal paragangliomas arise in a peripancreatic location and can manifest as primary pancreatic neoplasms .”
Nonepithelial Neoplasms of the Pancreas, Part 2: Malignant Tumors and Tumors of Uncertain Malignant Potential Maria A. Manning et al. RadioGraphics 2018; 38 (in press) - “Although paragangliomas are benign tumors, malignant transformation is unpredictable and can be definitively diagnosed only when metastases occur. As such, surgical excision is the main treatment of choice. Radiation therapy has also been used in patients who are not deemed operative candidates or in patients with malignant disease status after surgical resection.”
Nonepithelial Neoplasms of the Pancreas, Part 2: Malignant Tumors and Tumors of Uncertain Malignant Potential Maria A. Manning et al. RadioGraphics 2018; 38 (in press) - “The incidence of paraganglioma is the same in both men and women, and the tumor is most often detected between the ages of 42 and 85 years. Most retroperitoneal/pancreatic paragangliomas are nonfunctional and are discovered either incidentally or in a patient with abdominal pain or a palpable abdominal mass . Functioning paragangliomas can cause symptoms of catecholamine excess, including palpitations, headache, sweating, and hypertension.”
Nonepithelial Neoplasms of the Pancreas, Part 2: Malignant Tumors and Tumors of Uncertain Malignant Potential Maria A. Manning et al. RadioGraphics 2018; 38 (in press) - “Although uptake is not specific to paragangliomas, 18F-FDG PET/CT has high sensitivity (8%) for detection of the primary tumor and has shown significantly higher sensitivity than 123I-MIBG for detection of metastases. Recent studies have shown somatostatin receptor–based PET using gallium 68 (68Ga)–labeled DOTA (tetraazacyclododecane tetraacetic acid) peptides (68Ga-DOTATATE) to be far superior to all other functional imaging modalities, including 18F-FDG PET/CT, in detection of metastatic paraganglioma lesions, although the current clinical guidelines do not yet reflect these findings.”
Nonepithelial Neoplasms of the Pancreas, Part 2: Malignant Tumors and Tumors of Uncertain Malignant Potential Maria A. Manning et al. RadioGraphics 2018; 38 (in press) - “A correct diagnosis of paraganglioma is often difficult to make preoperatively. These hyperenhancing tumors will often be misdiagnosed as either pancreatic neuroendocrine tumors or less likely vascular lesions, such as aneurysms or pseudoaneurysms.”
Nonepithelial Neoplasms of the Pancreas, Part 2: Malignant Tumors and Tumors of Uncertain Malignant Potential Maria A. Manning et al. RadioGraphics 2018; 38 (in press)