CTICasesSDH-Mutation Related Multicentric Paragangliomas
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Imaging findings
Chest CT in a young patient shows two intensely contrast-enhancing, hypervascular masses located in classic mediastinal sites: one in the subaortic region and another in the right atrioventricular groove. A screening magnetic resonance cholangiopancreatography (MRCP) and HASTE sequence of the chest confirms corresponding, highly T2-hyperintense, vascular lesions.
Key takeaways
The presence of multiple, recurrent, or bilateral paragangliomas in a young patient strongly points to an underlying hereditary tumor syndrome, most commonly associated with mutations in the succinate dehydrogenase (SDH) gene subunits (e.g., SDHD or SDHB). Other syndromes in the differential diagnosis include MEN 2, neurofibromatosis type 1 (NF1), von Hippel-Lindau (VHL), and the Carney-Stratakis dyad or Carney triad. Because these tumors are highly vascular, contrast-enhanced CT and T2-weighted MRI are highly sensitive modalities for screening and mapping their locations.
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