SUN-908 Mediastinal Paraganglioma: A Rare Presentation of a Rare Tumor

• Published in: Journal of the Endocrine Society 2020-05, Vol.4 (Supplement_1)
• Main Authors: Deepika, Fnu, Andre, Karla Bermudez Saint, Kansara, Abhishek
• Format: Article
• Language: English
• Subjects: Tumor Biology
• ISSN: 2472-1972; 2472-1972
• DOI: 10.1210/jendso/bvaa046.562

BACKGROUND Paragangliomas (PPGL’s) are extra-adrenal chromaffin-cell originating tumors that arise from the para-aortic ganglia and are exceptionally rare making up only 1% of the mediastinal tumors. They are slowly growing and highly vascular tumors associated with high morbidity and mortality due to proximity and invasion into the heart, great vessels. The majority of them are asymptomatic and hence discovered incidentally. Surgical resection remains the standard of care with good long term survival. We describe a case of a young male with middle mediastinal PPGL who remained asymptomatic despite large functional tumor. CLINICAL CASE A 38-year old Arab male with no previous smoking history was referred to our hospital for evaluation of mediastinal mass that was incidentally noted on an echocardiogram done 1 year ago in his home country when he presented with intermittent exertional shortness of breath for 10 years. The workup done there with CT chest revealed a large mediastinal mass measuring 10.4×8.8×8.6 cm extending into ascending aorta, right pulmonary artery, right superior pulmonary vein, right and left atria as well as the main bronchi bilaterally invading the superior vena cava. However, he had no further treatment until he was seen 1 year later in the US at another institution where biochemical testing showed elevated plasma norepinephrine of 2697 (70-750 pg/ml, supine), dopamine 7667 (