Ganglioneuromas are benign, non-invasive tumors of the neural crest cells that give rise to the sympathetic nervous system. These tumors most commonly occur in the trunk; however, 1–5% occur in the head and neck . Cervical ganglioneuromas commonly present as slow-growing masses that cause compressive symptoms or as incidental findings on imaging. It has been reported that these tumors can cause symptoms related to catecholamine release; however, this was not present in our case nor in any reviewed cases of the last 15 years. Patients most commonly present in childhood or early adulthood, with management usually consisting of regular monitoring. Surgical resection is reserved for symptomatic patients. Recurrence is rare, with one incidence recorded in the literature. Resection is commonly complicated by self-limited postoperative HS due to disruption of the sympathetic cervical chain [8,9,10,11,12,13,14,15].
Here, we present a 7-year-old male with an initially asymptomatic neck mass that subsequently became associated with neck pain and odynophagia. The patient was found to have a parapharyngeal ganglioneuroma that was surgically excised, with the only complication being mild ipsilateral HS. This presentation and treatment outcome is consistent with other sympathetic chain parapharyngeal ganglioneuromas presented in the literature [8,9,10,11,12,13, 15]. This presentation is not universal, however varying based on the location of the lesion and the age of the patient.
In our review of the literature, there are 23 reported cases of ganglioneuroma affecting the head and neck. Sixteen of 23 occurred in patients under the age of 30 with a median age of 17, consistent with reported distributions of incidence . Of the 16 cases in patients under 30, 9 presented with a chief complaint of a neck mass, making it the most common presentation in that group (Table 1). Most of these presented in the absence of other symptoms. There were no cases of patients over 20 years of age presenting with a discernible neck mass [8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30]. Of the 23 reported cases reviewed, 7 presented with postoperative Horner’s syndrome (about 30%). Of those, 4/7 cases of postoperative Horner’s syndrome resolved [8,9,10,11,12,13, 15].
Interestingly, patients with ganglioneuromas in the para- or retropharyngeal spaces more commonly presented with an identifiable neck mass. In our review, 8 patients were found to have para- or retropharyngeal masses, with 5 of the 8 initially presenting with a neck mass. Two of the other patients presented with symptoms of compression: cough, neck pain, and stiffness. The third was discovered incidentally on imaging. The second most common subsite was prevertebral and cervical spine with 6 cases, with other reported cases in the submandibular space, external and internal auditory canals, uveal tract, base of the tongue, superior orbit, inferior jugular region, and the glossopharyngeal tract [8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30].
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