From Suspected Sepsis to Catecholamine Crisis: A Case of Functional Paraganglioma

Introduction: Paragangliomas are rare neuroendocrine tumors that originate from extra-adrenal chromaffin cells of the autonomic nervous system. These tumors may be non-functional or functional, with the latter capable of secreting catecholamines, leading to a range of symptoms including hypertension, tachycardia, and episodic headaches. We illustrate a case of a paraganglioma presenting as sepsis in the ICU.


Description: A 53-year-old female with a history of hypertension presented to the hospital with abdominal pain and vomiting for one day. In the emergency department, her blood pressure was 168/101 and heart rate was 142. She was given intravenous morphine for pain and intravenous fluids. She then became hypotensive despite fluid resuscitation and required norepinephrine. There was concern for septic shock, and she was started on antibiotics. She improved and was weaned off norepinephrine 24 hours after ICU admission. A CT of the abdomen revealed a 6.6 cm heterogeneously enhancing multilobulated presacral mass that was confirmed on MRI. Interventional radiology was unable to obtain a biopsy because there was no safe window, so it was excised without a biopsy. In the OR, the patient was hypertensive requiring a nicardipine infusion but became abruptly hypotensive requiring pressors once the mass was excised. Pathology revealed the mass was a paraganglioma causing intermittent catecholamine release and hypertension. The patient required several days in the ICU to wean off pressors.


Discussion: This case highlights a rare but critical presentation of a paraganglioma masquerading as septic shock. This patient developed profound hypotension with opiates in the ED due to both intrinsic hemodynamic effects and by potentially suppressing catecholamine release, but the response is unpredictable and requires careful monitoring. Further research is needed to clarify the precise risk and mechanisms in this specific population. The subsequent abrupt post-operative hypotension following tumor excision was the key physiologic response revealing the catecholamine-secreting nature of the tumor. This emphasizes the importance of considering paraganglioma in the differential diagnosis of retroperitoneal masses, especially when there is hemodynamic instability.