Metastatic Cardiac Paraganglioma: A Rare Subset of Mediastinal Paragangliomas
Journal Article - Embargoed Full-Text
59th Medical Wing San Antonio United States
Pagination or Media Count:
Liver biopsy confirmed the diagnosis of metastatic paraganglioma, specifically cardiac paraganglioma due to its primary location. Palpitations and tachycardia resolved with alpha and betaadrenergic blockade. Generally, first line treatment is surgery. Due to the presence of metastases at diagnosis, the patient is currently undergoing four cycles of neoadjuvant chemotherapy to improve the surgical outcome. Genetic testing, including SDHx evaluation, is pending. There are less than 200 reported cases of mediastinal paraganglioma in the literature, even less for cardiac paraganglioma as present in our patient. Dopamine secreting paragangliomas are rare and usually silent, leading to large tumor size and metastases prior to detection. Lack of clinical symptoms may be due to the vasodilatory effects of dopamine. There are only a handful of dopamine secreting mediastinal paragangliomas reported in the literature, but dopamine evaluation was useful in this case due to the minimal change in catecholamines and metanephrines and the negative plasma fractionated metanephrines.
- Medicine and Medical Research