Massive ganglioneuroma of the parapharyngeal space in a pediatric patient: a case report

• Published in: The Egyptian journal of otolaryngology 2022-12, Vol.38 (1), p.106-5, Article 106
• Main Authors: Jayavelu, Swetha, Anderson, Raleigh, Low, Garren, Asi, Karim, Roy, Soham
• Format: Article
• Language: English
• Subjects: Asymptomatic; Benign cervical mass; Carotid arteries; Case Report; Case reports; Children; Consent; Ganglioneuroma; Health aspects; Literature reviews; Medicine; Medicine & Public Health; Neck pain; Neuroblastic tumor; Neuroblastoma; Neurogenic tumor; Patients; Pediatric neck mass; Pediatrics; Surgery; Tumors
• ISSN: 1012-5574; 2090-8539
• DOI: 10.1186/s43163-022-00300-3

Background Neuroblastic tumors arise from primitive sympathetic ganglion cells and are the most common extracranial solid tumor of childhood. Ganglioneuroma is the most well-differentiated, benign subtype and comprises less than 1% of all soft tissue tumors. One to 5% of these occur in the head and neck, including the parapharyngeal space. It is uncommon to find a physically detectable, neoplastic parapharyngeal space mass in a child. Case report A 7-year-old boy presented with a painless left neck mass. Imaging revealed a 2.6 × 2.7 × 6.1 cm left parapharyngeal space mass. Fine-needle aspiration suggested a ganglioneuroma. The patient developed compressive symptoms, notably pain, thus the decision was made to surgically resect. The tumor was excised transcervically, preserving the great vessels, phrenic and cranial nerves. Postoperatively, the patient developed a mild ipsilateral Horner’s syndrome, suggesting the mass to be a ganglioneuroma of the sympathetic chain. Conclusions Cervical ganglioneuromas typically present as slow-growing masses that cause compressive symptoms or are found incidentally. Resection is reserved for those with significant symptoms and is often complicated by Horner’s syndrome. In the review of literature, there are 23 reported cases of ganglioneuroma in the head/neck with a median age of 17 years. Eight of these occurred in the para/retropharyngeal spaces. Of these, five presented as an asymptomatic neck mass, two presented with compressive symptoms, and one was discovered incidentally. It is important to consider ganglioneuroma in the workup of pediatric neck masses, even in young children. Asymptomatic masses should be monitored for symptomatic transformation, and excision should be considered.