SUN-279 "Benign Paraganglioma as a Cause of Catecholamine Surge: Diagnostic and Therapeutic Challenges"

Abstract Disclosure: B. Agrawal: None. H. Ali: None. H. Patel: None. K. pereira: None. A. imran: None. K.M. Doshi: None. Introduction: Paragangliomas are rare, highly vascular tumors arising from chromaffin cells of neural crest origin, with an annual incidence of 500 to 1600 cases in the United States. While non-functional paragangliomas are often incidental findings, functional tumors produce catecholamines, causing significant symptoms. Case Presentation: A 63-year-old male with uncontrolled hypertension and type 2 diabetes mellitus presented with three days of dyspnea and intermittent chest tightness. Initial evaluation revealed hypertension (220/108 mm Hg), mild troponin elevation, elevated calcium, hemoglobin A1c of 8%, and acute kidney injury (creatinine 1.4 mg/dL). Despite antihypertensive therapy, his blood pressure remained uncontrolled. Secondary hypertension workup showed mildly elevated parathyroid hormone (98.2 pg/mL) and significantly elevated blood and urine metanephrines (32 pg/mL and 305 μg/day), normetanephrines (3560 μg/day), and chromogranin A (2208 ng/mL). Imaging identified a 4.9-mm retroperitoneal mass below the renal arteries, inseparable from the inferior vena cava and pancreatic head. He was started on doxazosin and carvedilol before surgery. During resection, he experienced blood pressure fluctuations requiring pressor support. Postoperatively, he required nicardipine and nitroglycerin infusions for blood pressure control. Pathology confirmed a benign paraganglioma. By postoperative day 4, he was extubated and transitioned to oral antihypertensives. Follow-up plans included imaging and biochemical monitoring every 6 to 12 months. Discussion: Paragangliomas arise from parasympathetic and sympathetic ganglia, with most sympathetic tumors located in the thorax, abdomen, and pelvis. Approximately 30% are hereditary, often linked to succinate dehydrogenase gene mutations. Symptoms result from catecholamine excess and include anxiety, flushing, headaches, palpitations, and hypertension. The classic triad of headaches, sweating, and palpitations strongly suggests a secretory tumor. Diagnosis involves serum and urinary catecholamines and imaging such as CT, MRI, or MIBG scans. Preoperative α- and β-blockade reduces the risk of hypertensive crises. Surgery is the primary treatment, with intraoperative crises managed by agents like nitroprusside or nicardipine. Postoperative complications include hypoglycemia and hypotension, necessitating close monitoring. Long-term surveillance is crucial to detect recurrence or metastasis, with systemic therapy reserved for metastatic cases. Conclusion: Paragangliomas are rare but significant causes of secondary hypertension. Despite being benign, functional paragangliomas pose considerable intra- and postoperative risks, requiring meticulous management and lifelong follow-up to monitor for recurrence or metastasis. Presentation: Sunday, July 13, 2025

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Journal
Journal of the Endocrine Society
Published
2025-10-01
DOI
https://doi.org/10.1210/jendso/bvaf149.2481
Primary Topic
Adrenal and Paraganglionic Tumors
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article
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article

SUN-279 "Benign Paraganglioma as a Cause of Catecholamine Surge: Diagnostic and Therapeutic Challenges"

Bhavika Agrawal, Harshil Patel, Ali Hassan, Kesha Doshi et al.
Journal of the Endocrine Society
Adrenal and Paraganglionic Tumors
article

SUN-279 "Benign Paraganglioma as a Cause of Catecholamine Surge: Diagnostic and Therapeutic Challenges"

Bhavika Agrawal, Harshil Patel, Ali Hassan, Kesha Doshi, Kristal Pereira, Amin Imran
article en

Abstract

Abstract Disclosure: B. Agrawal: None. H. Ali: None. H. Patel: None. K. pereira: None. A. imran: None. K.M. Doshi: None. Introduction: Paragangliomas are rare, highly vascular tumors arising from chromaffin cells of neural crest origin, with an annual incidence of 500 to 1600 cases in the United States. While non-functional paragangliomas are often incidental findings, functional tumors produce catecholamines, causing significant symptoms. Case Presentation: A 63-year-old male with uncontrolled hypertension and type 2 diabetes mellitus presented with three days of dyspnea and intermittent chest tightness. Initial evaluation revealed hypertension (220/108 mm Hg), mild troponin elevation, elevated calcium, hemoglobin A1c of 8%, and acute kidney injury (creatinine 1.4 mg/dL). Despite antihypertensive therapy, his blood pressure remained uncontrolled. Secondary hypertension workup showed mildly elevated parathyroid hormone (98.2 pg/mL) and significantly elevated blood and urine metanephrines (32 pg/mL and 305 μg/day), normetanephrines (3560 μg/day), and chromogranin A (2208 ng/mL). Imaging identified a 4.9-mm retroperitoneal mass below the renal arteries, inseparable from the inferior vena cava and pancreatic head. He was started on doxazosin and carvedilol before surgery. During resection, he experienced blood pressure fluctuations requiring pressor support. Postoperatively, he required nicardipine and nitroglycerin infusions for blood pressure control. Pathology confirmed a benign paraganglioma. By postoperative day 4, he was extubated and transitioned to oral antihypertensives. Follow-up plans included imaging and biochemical monitoring every 6 to 12 months. Discussion: Paragangliomas arise from parasympathetic and sympathetic ganglia, with most sympathetic tumors located in the thorax, abdomen, and pelvis. Approximately 30% are hereditary, often linked to succinate dehydrogenase gene mutations. Symptoms result from catecholamine excess and include anxiety, flushing, headaches, palpitations, and hypertension. The classic triad of headaches, sweating, and palpitations strongly suggests a secretory tumor. Diagnosis involves serum and urinary catecholamines and imaging such as CT, MRI, or MIBG scans. Preoperative α- and β-blockade reduces the risk of hypertensive crises. Surgery is the primary treatment, with intraoperative crises managed by agents like nitroprusside or nicardipine. Postoperative complications include hypoglycemia and hypotension, necessitating close monitoring. Long-term surveillance is crucial to detect recurrence or metastasis, with systemic therapy reserved for metastatic cases. Conclusion: Paragangliomas are rare but significant causes of secondary hypertension. Despite being benign, functional paragangliomas pose considerable intra- and postoperative risks, requiring meticulous management and lifelong follow-up to monitor for recurrence or metastasis. Presentation: Sunday, July 13, 2025

Journal of the Endocrine SocietyVol. 9(Supplement_1)
Mercy Fitzgerald Hospital (US), B.J. Medical College (IN)
Openalex Percentile: Top 52%
Adrenal and Paraganglionic Tumors
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