Abdominal paraganglioma in a patient with fever of unknown origin

Abstract

Introduction

Paragangliomas have a wide range of clinical presentations. A significant portion of these tumors are discovered as abdominal masses on imaging or palpation in patients without typical symptoms of catecholamine excess [1]. This case reports a 28-year-old female who presented with fever of unknown origin and was found to have an abdominal mass on CT imaging. This mass was biopsied prior to a complete evaluation and confirmed to be a paraganglioma.

Case report

We discuss the case of a 28 year-old-female who presented to the Endocrinology office after she was diagnosed with a paraganglioma on retroperitoneal biopsy. The patient had initially presented to her primary care provider with fever of unknown origin. She was found to have a large retroperitoneal mass on a non-contrast CT scan done for infectious work-up, which prompted referrals to Medical Oncology and Surgery. A contrast-enhanced CT scan was ordered to assess for metastatic disease and re-demonstrated the retroperitoneal mass. It also provided further information, specifying that the mass was most suspicious for a paraganglioma. Despite this, the patient underwent a biopsy that confirmed this diagnosis without further evaluation prior. She was then referred to Endocrinology and biochemical testing showed mildly elevated normetanephrine on urine and blood analyses. Successful surgical excision of the mass was completed with preceding alpha-blockade. Genetic testing was negative.

Discussion

Paragangliomas have diverse clinical presentations, which can make diagnosis challenging. Fever of unknown origin, as seen in this case, represents a rare manifestation [2,3]. The accuracy of CT scans for identifying paragangliomas has increased significantly over the last decade [4]. Biopsy of these massesis associated with significant morbidity. It is important that clinicians are aware that paragangliomas may be present in the absence of hypertension or classic symptoms associated with pheochromocytomas.

Conclusion

Paragangliomas can be discovered as abdominal masses on imaging in patients without typical symptoms of catecholamine hyper-secretion. If a radiology report suggests a high likelihood of a paraganglioma it is critical to delay a potentially high-risk biopsy until further evaluation can be completed.