Malignant hypertension (MH) is a medical emergency rarely associated with paraganglioma. We present a unique case of a MH associated with a paraganglioma of the tonsil.
A 29 year-old man presented with mental status and visual changes, hypertension (BP 220/120 torr), and respiratory failure. During intubation for respiratory failure, a mass in the right tonsillar region was found. The MH required intravenous labetalol and phenoxybenzamine. A neck MRI showed a 2 x 2.5 x 4cm right tonsillar mass sparing the parapharyngeal fat. CT scans of the chest, abdomen, and pelvis were normal. A biopsy of the mass showed prominent fibrovascular stroma (classic “zellballen” pattern) consistent with a paraganglioma. Plasma catecholamines were elevated and remained high after clonidine administration. Surgical resection via transoral route with vascular control within the neck was performed following embolization of the tumor. The pathology confirmed paraganglioma. His hypertension was more easily managed following its resection. He was extubated three days after surgery and subsequently transferred to a rehabilitation facility ten days later. His blood pressure was 160/90 torr.DISCUSSION: This case of malignant hypertension is unique in its relationship to a catecholamine secreting paraganglioma. The paraganglioma location (tonsil) has not previously been reported in the literature. Malignant hypertension is a medical emergency usually occurring secondary to uncontrolled hypertension. Rare causes of MH include pheochromocytoma and paraganglioma. Paragangliomas often present as painless upper neck masses. Hypertension, headache, palpitations and sweating may occur; however, functional hormone secretion is uncommon when the tumor arises in the head and neck region (2 percent). This tumor secreted catecholamines producing MH manifested as CNS and respiratory dysfunction. No laboratory evidence of pheochromocytoma was found. Resection of the paraganglioma resulted in a significant improvement in blood pressure control.
This unique case shows the association of MH with a catecholamine secreting paraganlioma of the tonsillar bed. Recognition of this entity as a potential cause for MH is important so that appropriate management is performed.
S. Yakoob, None.