An IR-guided para-aortic lymph node biopsy in a 68-year-old male triggered a catastrophic catecholamine surge secondary to an undiagnosed paraganglioma, leading to Takotsubo cardiomyopathy.
Case Report (n=1)
Invasive procedures like needle biopsies in patients with undiagnosed paraganglioma can trigger massive catecholamine release leading to life-threatening complications such as Takotsubo cardiomyopathy.
Abstract Introduction Pheochromocytoma is a rare neuroendocrine tumor arising from chromaffin cell of the adrenal medulla, or from the extra-adrenal paraganglia (paraganglioma).It has an annual incidence of 0.6 cases per 100000 individuals and occurs sporadically or as part of hereditary syndromes (e.g., MEN 2, NF 1, or VHL) in up to 40% of cases. These tumors are characterized by the excessive secretion of catecholamines, which produces a wide array of presentations, classically as the triad of headache, palpitations, and episodic sweating. Diagnosis is confirmed through elevated plasma or 24-hour urine metanephrines and localized with CT or MRI. We present an unusual case of a patient who was incidentally found to have a paraganglioma that triggered a catecholamine surge following a para-aortic lymph node biopsy. Case Report A 68-year-old male with a history of hypertension, type 2 diabetes, HIV, and polysubstance use presented with acute hypoxic respiratory failure and circulatory overload. Vitals were notable for heart rate 117 bpm, blood pressure 131/87 mm Hg, respiratory rate 29/minute, SpO2 90% on BiPAP. Labs were notable for Cr 12 (baseline 1.1), BUN 101 ,K 6.1. BNP 3,744. Chest X-ray demonstrated cardiomegaly with interstitial edema and small left pleural effusion. The patient’s acute renal failure was likely due to acute tubular necrosis from cocaine use, which progressed to uremic encephalopathy requiring emergent CRRT. He had persistent abdominal pain which prompted a CT A/P, demonstrating a 3.3 cm para-aortic lymph node. Due to concerns for malignancy, the patient underwent an IR-guided lymph node biopsy. Immediately post-procedure, he became acutely hypertensive, tachycardic, and tachypneic, developing profound metabolic acidosis with a lactate 17 requiring ICU admission. An echocardiogram revealed newly depressed LVEF of 25%, grade III diastolic dysfunction, and severe tricuspid regurgitation suggestive of catecholamine-induced cardiomyopathy. Given his guarded prognosis, the patient opted for comfort measures. Core biopsy confirmed the diagnosis, showing epithelioid chief cells positive for S-100, synaptophysin, INSM1, and GATA3. Discussion Pheochromocytoma and paraganglioma have a broad clinical spectrum, with 10-15% of cases occurring in extra-adrenal locations. The potential for dramatic hemodynamic instability including hypertensive crisis, arrhythmias, cardiomyopathy, and multi-organ damage due to excessive catecholamine release underscores the need for a high index of suspicion before any invasive procedure. Procedures such as needle biopsies can mechanically trigger the massive release of stored hormones, leading to these crises. Preoperative preparation, typically involving alpha-adrenergic blockade, with or without beta-blockade, is mandatory to prevent these life-threatening complications. This abstract is funded by: None
Rajagopal et al. (Fri,) conducted a case report in Paraganglioma and Takotsubo Cardiomyopathy (n=1). IR-guided lymph node biopsy was evaluated. An IR-guided para-aortic lymph node biopsy in a 68-year-old male triggered a catastrophic catecholamine surge secondary to an undiagnosed paraganglioma, leading to Takotsubo cardiomyopathy.
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