Hypertensive retinopathy as the first manifestation of extra-adrenal paraganglioma

Paragangliomas are rare neuroendocrine tumors originating from paraganglionic cells in the autonomic nervous system and which are capable of secreting catecholamines and causing hypertensive crises. Although typically benign, they can exhibit aggressive features and present with varied clinical manifestations depending on location and catecholamine activity. Herein, we report an unusual case of a 28-year-old male who initially presented with bilateral grade IV hypertensive retinopathy, an uncommon initial manifestation of paraganglioma. Fundus examination revealed severe hypertensive retinopathy with exudative retinal detachment. Laboratory evaluation demonstrated markedly elevated plasma and urinary catecholamines and metanephrines. Imaging, including computed tomography (CT) and iodine-123 metaiodobenzylguanidine (MIBG) scintigraphy, confirmed a highly vascularized mass near the left adrenal gland, consistent with extra-adrenal paraganglioma. The patient's hypertension was medically stabilized prior to successful surgical tumor resection. Postoperatively, blood pressure normalized without medication, and significant improvement was observed in retinal findings, including resolution of disc edema and macular exudates. This case highlights the importance of considering paraganglioma in the differential diagnosis of malignant hypertension with ocular involvement, even when typical systemic symptoms of catecholamine excess are absent. Prompt recognition and intervention can lead to favorable visual and systemic outcomes.