Carotid Body Paraganglioma Extending to the Middle Ear Cavity
A 51-year-old woman presented with a remote history of surgery for pheochromocytoma and received surgery 20 years ago. She presented with a left neck mass (Fig. 1A) and left-sided pulsatile tinnitus for 2 years. She also felt disoriented and lightheaded every time she turned her head to the left. Otoscopy revealed a left-sided, red, pulsatile middle ear mass (Fig. 1B, Video, Supplementary Digital Content 1, http://links.lww.com/MAO/A565). In addition, left-sided intermittent facial weakness with involuntary left facial twitching (Video, Supplementary Digital Content 2, http://links.lww.com/MAO/A566) was observed. The patient could completely close her left eye by exerting minimal effort, and forehead motion was satisfactory (House-Brackmann grade II). High-resolution computed tomography revealed a soft tissue lesion in the middle ear without bony destruction (Fig. 1D, arrow). The relationship between the left jugular bulb and temporal bone was also revealed in the image (Fig. 1C, arrow). A T1-weighted magnetic resonance image enhanced with gadolinium contrast revealed a carotid body paraganglioma occupying the bifurcation of the common carotid artery with extension to the left-sided middle ear cavity (Fig. 2).
Glomus tumors of the head and neck are associated with four primary locations: the jugular bulb (glomus jugulare), middle ear cavity (glomus tympanicum), vagus nerve (glomus vagale), and carotid body (carotid body tumor) (1,2). Because glomus tumors generally appear as solitary lesions at one site, lesion extension to other sites is rare (3). Our case represents an unusual example of a paraganglioma with involvement of both the carotid body and the ipsilateral middle ear. A carotid body paraganglioma often has a specific characteristic: these tumors often “splay” the carotid artery bifurcation, which is called the “Lyre” sign (Figs. 2, B and D). The middle ear component of the tumor may be responsible for the involvement of the facial nerve, causing paresis and hemifacial spasm (4). The differential diagnosis of head and neck paragangliomas primarily depends on the actual location of the lesion. The differential diagnoses of tumors occurring in the jugulotympanic area include middle ear adenoma, meningioma, high jugular bulb, and schwannoma.
This patient had a pheochromocytoma previously, and at the time of this study, she presented with a paraganglioma of the head and neck. Thus, we assumed that the patient may have had paraganglioma–pheochromocytoma syndrome, which is a rare disease caused by RET, VHL, NF1, SDHB, SDHC, and SDHD gene mutations with overproduction of catecholamines, as previously described by Toutounchi et al. (5).
By using current techniques, carotid body and vagal body paragangliomas can typically be managed by performing surgery alone. Other head and neck paragangliomas can be treated with a combination of surgery and radiation or radiation alone, based on size and location. The patient in this study was followed up for 1 year without surgical intervention or radiation therapy. As of the submission of this report, tumor size has remained stable and the patient continues to have a mild facial weakness.