Endocrine Abstracts

Introduction

Paragangliomas (PGs) are rare tumors arising from sympathetic and parasympathetic paraganglia with an incidence of 15–20%. We report a case of a pre-operative diagnosis of retroperitoneal PGs evoked on anatomic imaging confirmed by biochemical evaluation.

Observation

A 55-year-old female was referred to the endocrinology department for investigation of a paroxistic hypertension. For her personal medical history, she had a 15-year history of hypertension with hypertrophic obstructive cardiomyopathy (HCM). She was treated with calcium channel blockers, and angiotensin 2 receptor blockers but her hypertension was never controlled. She was receiving high doses of propranolol for her HCM. An abdominal computed tomographic (CT) scan done 3 years ago for abdominal pain revealed retroperitoneal mass; lymphoma was suspected and a biopsy was indicated but the patient was lost to follow-up. There was no significant family history. The patient complained of paroxysms of headache, sweating and palpitations associated with abdominal pain for over 15 years. On examination, she had a blood pressure of 220/120 mmHg with orthostatic hypotension. 24-h blood pressure measurement has confirmed the presence of paroxism up to 270/140 mmHg. Biochemical testing revealed an elevated plasma normetanephrines with normal metanephrines level. The abdominal CT examination revealed two left hypervascular retroperitoneal masses (43 mm and 65 mm) with left adrenal gland and abdominal aorta contact, consistent with PGs. MIBG scintigraphy revealed an uptake in the retroperitoneal space corresponding to the location of the masses. On the basis of all findings, the diagnosis of functional retroperitoneal non-metastatic PGs is made. Surgery is postponed after septal alcoholization for HCM.

Conclusion

Our case highlights the importance of including extra-adrenal PGs in the differential diagnosis of retroperitoneal tumors, despite their rarity. Definitive diagnosis requires a histopathological assessment and genetic testing as PGs were detected before the age of 50 years.