A Case of Functional Para-Aortic Paraganglioma Causing Accelerated Hypertension in an HIV-Positive Patient With...

Functional retroperitoneal paragangliomas are rare neuroendocrine tumors that typically present with catecholamine-mediated hypertension. 68Ga-DOTATATE PET/CT is widely used as a preferred functional imaging modality for the localization of pheochromocytoma and paraganglioma (PPGL), though false-negative results may occur. The coexistence of paraganglioma and human immunodeficiency virus (HIV) infection is exceptionally rare, with no prior reports of functional retroperitoneal paraganglioma in HIV-positive patients. We report a 42-year-old female living with HIV on stable antiretroviral therapy who presented with accelerated hypertension. Biochemical evaluation confirmed catecholamine excess with elevated plasma metanephrines. Contrast-enhanced CT demonstrated a 5 × 5 cm left para-aortic mass. Despite strong biochemical and anatomical evidence, 68Ga-DOTATATE PET/CT showed minimal uptake at the lesion site. Following comprehensive preoperative alpha-adrenergic blockade and multidisciplinary optimization, the patient underwent successful laparoscopic excision of the mass. Histopathology confirmed paraganglioma with insulinoma-associated protein 1 (INSM1)-positive staining. Postoperatively, blood pressure normalized, and biochemical markers showed complete resolution. The patient remained disease-free at six-month follow-up with excellent HIV virologic control. This case represents, to our knowledge, the first reported functional retroperitoneal paraganglioma in an HIV-positive patient. It underscores that DOTATATE-negative imaging should not exclude the diagnosis when biochemical and anatomical evidence is compelling. Laparoscopic resection is feasible and safe in carefully selected patients with well-controlled HIV infection, provided meticulous perioperative preparation and hemodynamic management are implemented.