CVICU Medical Director Mercy Hospital Saint Louis, Missouri, United States
Introduction: Hypertensive emergency is a critical condition requiring prompt intervention. While common causes include chronic hypertension and renal disease, it is essential to consider less common etiologies in young patients. Extra-adrenal paragangliomas are rare neuroendocrine tumors originating from chromaffin cells, associated with significant morbidity due to catecholamine secretion. This case discusses a young female who presented with hypertensive emergency, ultimately diagnosed with retroperitoneal paraganglioma.
Description: An 18-year-old female with no prior medical or family history due to her adoption was admitted with hypertensive emergency and respiratory failure requiring intubation. Initial presentation included a blood pressure of 255/200 and supraventricular tachycardia. Bedside ultrasound indicated a reduced ejection fraction of 35%. A CT chest showed pulmonary edema. The patient exhibited acute kidney injury with a serum creatinine level of 3.0 mg/dL. The patient’s respiratory status improved significantly after achieving blood pressure control, allowing for extubation to room air. A repeat echocardiogram demonstrated an improved ejection fraction of 55%. A more detailed history revealed that the patient had been experiencing episodes of dizziness, flushing, and fatigue upon physical exertion. An MRI revealed a 6.6 cm retroperitoneal mass located between the aorta, inferior vena cava, and portal vein, with characteristics suggestive of paraganglioma. Laboratory analysis revealed a four-fold elevation in urinary metanephrines. Genetic testing identified an SDHB gene mutation, which is associated with hereditary paragangliomas and high risk of malignancy. The patient was initiated on labetalol and doxazosin therapy. Follow-up care was arranged with a specialized center and she was discharged home.
Discussion: Paragangliomas are rare, accounting for 0.05-0.1% of patients with hypertension. This case highlights the importance of considering paraganglioma in young patients presenting with hypertensive emergencies. Mutations in the succinate hydrogenase complex is a well known etiology of paragangliomas. Early recognition and appropriate referral to a high volume surgical center are crucial for optimizing patient outcomes, especially in cases with potential malignant ramifications.
