Clinical Progression of a Paraganglioma Over Many Years in a Man With Congenital Heart Disease
Background: Documented symptomatic progression of a paraganglioma (PGL) over many years is unusual. Our objective is to report a young man with such an occurrence. Case Report: A 27-year-old male presented with headache, sweating, and palpitation. He had a history of cyanotic congenital heart diseas...
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| Format: | Article |
| Language: | English |
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Elsevier
2023-11-01
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| Series: | AACE Clinical Case Reports |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2376060523001396 |
| _version_ | 1797526217315844096 |
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| author | Megan M. McConnell, MD Maralee R. Kanin, MD Martin S. Auerbach, MD Run Yu, MD, PhD |
| author_facet | Megan M. McConnell, MD Maralee R. Kanin, MD Martin S. Auerbach, MD Run Yu, MD, PhD |
| author_sort | Megan M. McConnell, MD |
| collection | DOAJ |
| description | Background: Documented symptomatic progression of a paraganglioma (PGL) over many years is unusual. Our objective is to report a young man with such an occurrence. Case Report: A 27-year-old male presented with headache, sweating, and palpitation. He had a history of cyanotic congenital heart disease. Five years before presentation, he had 24-hour urine metanephrines 43 mcg/d (25-222), vanillylmandelic acid 3 mg/d (<6), and homovanillic acid 2.4 mg/d (1.6-7.5) levels and a 3.13 cm mass in the upper aortocaval space. Subsequent imaging showed slow growth of the mass. On admission, his blood pressure was 197/134 mm Hg, heart rate was 163 beats per minute, respiratory rate was 25 per minute, and oxygen saturation was 76% on room air. His 24-hour urine normetanephrine level was 2644 mcg/d (81-667) while metanephrine was 405 mcg/d (55-320). Plasma free metanephrine level was 0.92 nmol/L (0-0.49) and normetanephrine was 11.85 nmol/L (0-0.89). DOTATATE positron emission tomography–computed tomography revealed a 4.3 × 3.1 × 4.9 cm mass with activity in the right upper aortocaval space. He was treated with Prazosin. Two months later, he underwent resection of the mass. Pathology diagnosed a 4.9 cm PGL. He had improvement in metanephrine levels. Discussion: PGL is diagnosed by documenting excess catecholamines and identifying a lesion on imaging. False negative laboratory testing is rare but can occur. Patients with cyanotic congenital heart disease have a greater risk of developing PGL. Conclusion: It is crucial to evaluate a patient for PGL if clinical conditions suggest catecholamine excess, especially if a retroperitoneal tumor has grown or the patient has risk factors. |
| first_indexed | 2024-03-10T09:27:05Z |
| format | Article |
| id | doaj.art-e0443628a7e6424db56ef5abca30ece6 |
| institution | Directory Open Access Journal |
| issn | 2376-0605 |
| language | English |
| last_indexed | 2024-03-10T09:27:05Z |
| publishDate | 2023-11-01 |
| publisher | Elsevier |
| record_format | Article |
| series | AACE Clinical Case Reports |
| spelling | doaj.art-e0443628a7e6424db56ef5abca30ece62023-11-22T04:47:52ZengElsevierAACE Clinical Case Reports2376-06052023-11-0196193196Clinical Progression of a Paraganglioma Over Many Years in a Man With Congenital Heart DiseaseMegan M. McConnell, MD0Maralee R. Kanin, MD1Martin S. Auerbach, MD2Run Yu, MD, PhD3Division of Endocrinology, Diabetes & Metabolism, Department of Internal Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California; Address correspondence to Dr Megan McConnell, 10833 Le Conte Ave, CHS 57-145, Los Angeles, California 90095.Division of Endocrinology, Diabetes & Metabolism, Department of Internal Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CaliforniaDepartment of Nuclear Medicine and Department of Molecular and Medical Pharmacology, David Geffen School of Medicine at UCLA, Los Angeles, CaliforniaDivision of Endocrinology, Diabetes & Metabolism, Department of Internal Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CaliforniaBackground: Documented symptomatic progression of a paraganglioma (PGL) over many years is unusual. Our objective is to report a young man with such an occurrence. Case Report: A 27-year-old male presented with headache, sweating, and palpitation. He had a history of cyanotic congenital heart disease. Five years before presentation, he had 24-hour urine metanephrines 43 mcg/d (25-222), vanillylmandelic acid 3 mg/d (<6), and homovanillic acid 2.4 mg/d (1.6-7.5) levels and a 3.13 cm mass in the upper aortocaval space. Subsequent imaging showed slow growth of the mass. On admission, his blood pressure was 197/134 mm Hg, heart rate was 163 beats per minute, respiratory rate was 25 per minute, and oxygen saturation was 76% on room air. His 24-hour urine normetanephrine level was 2644 mcg/d (81-667) while metanephrine was 405 mcg/d (55-320). Plasma free metanephrine level was 0.92 nmol/L (0-0.49) and normetanephrine was 11.85 nmol/L (0-0.89). DOTATATE positron emission tomography–computed tomography revealed a 4.3 × 3.1 × 4.9 cm mass with activity in the right upper aortocaval space. He was treated with Prazosin. Two months later, he underwent resection of the mass. Pathology diagnosed a 4.9 cm PGL. He had improvement in metanephrine levels. Discussion: PGL is diagnosed by documenting excess catecholamines and identifying a lesion on imaging. False negative laboratory testing is rare but can occur. Patients with cyanotic congenital heart disease have a greater risk of developing PGL. Conclusion: It is crucial to evaluate a patient for PGL if clinical conditions suggest catecholamine excess, especially if a retroperitoneal tumor has grown or the patient has risk factors.http://www.sciencedirect.com/science/article/pii/S2376060523001396pheochromocytomaparagangliomaparaganglioma in congenital cyanotic heart disease |
| spellingShingle | Megan M. McConnell, MD Maralee R. Kanin, MD Martin S. Auerbach, MD Run Yu, MD, PhD Clinical Progression of a Paraganglioma Over Many Years in a Man With Congenital Heart Disease AACE Clinical Case Reports pheochromocytoma paraganglioma paraganglioma in congenital cyanotic heart disease |
| title | Clinical Progression of a Paraganglioma Over Many Years in a Man With Congenital Heart Disease |
| title_full | Clinical Progression of a Paraganglioma Over Many Years in a Man With Congenital Heart Disease |
| title_fullStr | Clinical Progression of a Paraganglioma Over Many Years in a Man With Congenital Heart Disease |
| title_full_unstemmed | Clinical Progression of a Paraganglioma Over Many Years in a Man With Congenital Heart Disease |
| title_short | Clinical Progression of a Paraganglioma Over Many Years in a Man With Congenital Heart Disease |
| title_sort | clinical progression of a paraganglioma over many years in a man with congenital heart disease |
| topic | pheochromocytoma paraganglioma paraganglioma in congenital cyanotic heart disease |
| url | http://www.sciencedirect.com/science/article/pii/S2376060523001396 |
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