Paraganglioma at the cerebellopontine angle: A case report and review of literature

Abstract

Paragangliomas (PGLs) are rare neuroendocrine tumors. Sometimes, these tumors secrete excessive catecholamines, which results in the manifestations of various signs and symptoms, usually with a triad of hypertension, tachycardia, and headache. We report the case of a 42-year-old woman presenting with uncontrolled hypertension, right facial palsy, vomiting, and disturbed gait. Diagnosis for PGL was confirmed on postoperative histological examination of the excised mass and correlated with preoperative clinical and radiological findings. Tumor excision was done via a suboccipital craniotomy approach. Our case presents the typically severe features of a jugulotympanic PGL, but most importantly, it highlights the necessity of biochemical diagnosing, thorough probing of the causes of hypertension, and a multi-disciplinary approach in dealing with these tumors. Moreover, the case emphasizes necessitating the use of preoperative embolization in vascular tumors of the head and neck to avoid a hemorrhagic crisis during surgery. Unfortunately, due to a lack of adequate hospital funds, the surgeon had to proceed without preoperative embolization. Despite such a risk, the excision was a success.

Keywords: catecholamine secreting tumor; cerebellopontine angle; extra‐adrenal paraganglioma; head and neck neoplasms; hypertension; hypoxia‐inducible factor (HIF)‐1α.

FIGURE 1

Pre‐operative contrast‐enhanced magnetic resonance imaging (MRI) brain (axial section). Arrow pointing toward a well‐demarcated tumorous mass at right cerebellopontine angle (CPA) with typical salt and pepper appearance.

FIGURE 2

Pre‐operative contrast‐enhanced magnetic resonance imaging (MRI) brain (sagittal section). Arrow points to the tumor with intense post‐contrast enhancement at the cerebellopontine angle (CPA). Its extension into the right parapharyngeal space and right internal auditory canal can also be seen.

FIGURE 3

Post‐operative non‐contrast enhanced computerized tomography (CT)‐brain (axial section). Arrows indicate successful tumor resection in the brain. Surgical intervention has removed the neoplastic lesion, indicated by the absence of abnormal tissue density. This scan confirmed the achievement of near‐complete tumor excision.