Hypotension in a woman with a metastatic dopamine-secreting carotid body tumor.
OBJECTIVE
To describe a woman with metastatic carotid body tumor in whom hypotension occurred in the setting of exceedingly high plasma dopamine levels.


METHODS
We present a case report and review the literature on the topic of dopamine-secreting paraganglioma or pheochromocytoma.


RESULTS
A previously healthy 40-year-old Asian woman noted difficulty with swallowing and hoarseness. No neck mass was visible, and she had no symptoms of catecholamine excess and no family history of endocrine disorders or malignant disease. Indirect laryngoscopy revealed a paralyzed left vocal cord and a nonulcerating mass in the left parapharyngeal space. An initial needle biopsy was interpreted as undifferentiated carcinoma. After a second biopsy, this mass was diagnosed as a neuroendocrine tumor, consistent with paraganglioma. The patient underwent surgical resection and radiation therapy (total dose, 40 Gy), after which she remained asymptomatic for 11 years. Then loss of weight, fatigue, nausea, and hypotensive episodes (blood pressures as low as 70/35 mm Hg) prompted whole-body imaging with bone scans, computed tomography, and magnetic resonance imaging, which disclosed several lesions in the liver, lungs, and spine, suggestive of metastatic disease. The adrenal glands were unremarkable. A metaiodobenzylguanidine scan with use of (131)I was negative. Liver biopsy of a hypodense lesion revealed a neuroendocrine tumor by histologic and immunohistochemical studies. Because of the patient's history, malignant paraganglioma was diagnosed. The tumor secreted predominantly dopamine at extraordinary levels (plasma concentration 27,942 pg/mL; normal, <30). The patient died before further treatment could be initiated.


CONCLUSIONS
Carotid body tumors usually do not secrete catecholamines but frequently metastasize. During progression, these neuroendocrine tumors may become able to produce and secrete selected catecholamines such as dopamine. Dopamine can lower the blood pressure rather than causing hypertension, even though hypertension is one of the main symptoms of a pheochromocytoma.
Koch
Christian A
CA
National Institute of Child Health and Human Development, Pediatric and Reproductive Endocrinology Branch. National Institutes of Health, Bethesda, Maryland, USA.
Rodbard
Joseph S
JS
Brouwers
Frederieke M
FM
Eisenhofer
Graeme
G
Pacak
Karel
K
D019797:3-Iodobenzylguanidine; D000328:Adult; D002345:Carotid Body Tumor / Q000150:complications* / Q000000981:diagnostic imaging / Q000378:metabolism*; D002395:Catecholamines / Q000097:blood; D004298:Dopamine / Q000378:metabolism*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007022:Hypotension / Q000209:etiology* / Q000503:physiopathology; D009362:Neoplasm Metastasis; D010235:Paraganglioma / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology; D010673:Pheochromocytoma / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology; D011877:Radionuclide Imaging; D019275:Radiopharmaceuticals; D014057:Tomography, X-Ray Computed
D002395:Catecholamines; D019275:Radiopharmaceuticals; D019797:3-Iodobenzylguanidine; D004298:Dopamine