Abstract | OBJECTIVE: METHODS: 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. CONCLUSION:
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Authors | Christian A Koch, Joseph S Rodbard, Frederieke M Brouwers, Graeme Eisenhofer, Karel Pacak |
Journal | Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists
(Endocr Pract)
2003 Jul-Aug
Vol. 9
Issue 4
Pg. 310-4
ISSN: 1530-891X [Print] United States |
PMID | 14561577
(Publication Type: Case Reports, Journal Article)
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Chemical References |
- Catecholamines
- Radiopharmaceuticals
- 3-Iodobenzylguanidine
- Dopamine
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Topics |
- 3-Iodobenzylguanidine
- Adult
- Carotid Body Tumor
(complications, diagnostic imaging, metabolism)
- Catecholamines
(blood)
- Dopamine
(metabolism)
- Fatal Outcome
- Female
- Humans
- Hypotension
(etiology, physiopathology)
- Neoplasm Metastasis
- Paraganglioma
(complications, diagnostic imaging, pathology)
- Pheochromocytoma
(complications, diagnostic imaging, pathology)
- Radionuclide Imaging
- Radiopharmaceuticals
- Tomography, X-Ray Computed
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