Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Mediastinal paraganglioma
- Radiologic Findings
- Fig 1. On the PA chest radiograph, a mediastinal bulging contour is noted, causing loss of the normal aortopulmonary window silhouette.
Fig 2. On contrast-enhanced chest CT, there is an intensely and heterogeneously enhancing soft-tissue mass with an internal necrotic area and prominently engorged peripheral vascular structures in the left paratracheal area of the middle mediastinum.
Fig 3. On MRI, a mass shows mixed high and low signal intensities on both T2-weighted and T1-weighted images, with internal T2 signal voids suggestive of high-flow vessels. Gadolinium-enhanced T1-weighted images demonstrate heterogeneous enhancement with suspected internal necrosis.
Fig 4. On PET-CT, the mass demonstrates hypermetabolism.
- Brief Review
- Paragangliomas are rare neuroendocrine tumors of extra-adrenal paraganglionic tissue and are indistinguishable from pheochromocytomas at the cellular level. They manifest clinically with mass effect, or symptoms of catecholamine excess such as episodic hypertension, tachycardia, palpitations, or diaphoresis.
Paragangliomas are usually located in the abdomen or skull base and neck area, along distributions of the sympathetic and parasympathetic ganglia, respectively. About 2% of paragangliomas are found in the mediastinum, where they represent a small percentage of mediastinal masses.
Mediastinal paragangliomas typically appear as intensely and heterogeneously enhancing hypervascular masses located adjacent to the airways, major vessels, or the heart. Due to their rich vascularity, they show marked enhancement after intravenous contrast administration. On MRI, high-velocity flow within intratumoral vessels produces the characteristic “salt-and-pepper” appearance, with flow voids on T2-weighted images. Additional imaging features may include cystic change, necrosis, intratumoral hemorrhage, and calcification.
The differential diagnosis of a hypervascular mediastinal mass is limited and includes vascular malformations, thymic carcinoids, Castleman disease, and certain metastatic tumors such as renal cell carcinoma. Chest CT with intravenous contrast is effective in diagnosing paragangliomas of the mediastinum. MRI or radioisotope whole body imaging such as the MIBG scintigraphy and Gallium 68 (68-Ga) 1,4,7,10-tetraazacyclododecane-1,4,7,10-tetraacetic acid (DOTA)-octreotate (DOTATATE) positron emission tomography (PET), can often help distinguish paragangliomas from other hypervascular mediastinal masses and help locate paragangliomas elsewhere in the body. In addition, delayed Gadolinium enhancement on MRI can provide valuable presurgical information by detecting tumor encapsulation and tissue invasion.
Surgical resection remains the preferred method of treatment for localized mediastinal paragangliomas, especially when symptomatic. Radiation therapy has also been used for advanced or unresectable tumor.
- References
- 1. Kanj AN, Young WF, Ryu JH. Mediastinal Paraganglioma: A retrospective analysis of 51 cases. Respir Med. 2023;216:107296.
- Keywords