Paediatric parapharyngeal ganglioneuroma

A 30-year-old woman was referred to the ear, nose and throat clinic by her primary care physician for a 10-year history of an asymptomatic, large, right-sided neck mass. On examination, the patient had a palpable, non-tender, five-by-four centimetre, mobile, right-sided level II neck mass. CT scan with intravenous contrast showed an enhancing mass with multiple fluid cavities, splaying the external carotid and internal carotid arteries, concerning for a carotid body tumour. Patient was then referred to interventional radiology for angiography and embolisation prior to definitive surgical excision. However, when the mass was then excised surgically, final pathology identified the mass as a ganglioneuroma. Patient recovered well postoperatively with some ptosis of the right eye and symptoms consistent with first bite syndrome, treated with conservative measures.

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Cervical ganglioneuroma mimicking a carotid body tumour

Britten

Entezami

Chang

2020

BMJ Case Rep

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Parapharyngeal Space Ganglioneuroma: Clinical Experience and Review of the Literature

Feng

Liao

et al. 2022

Ear Nose Throat J

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Ganglioneuroma is a rare benign tumor originating in the sympathetic ganglia, composed of differentiated ganglion cells, nerve sheath cells, and nerve fibers, which tend to occur in the posterior mediastinum, adrenal gland, retroperitoneal, and other locations, occurring in the head and neck is relatively rare, and parapharyngeal space involvement is extremely rare. In our report, we present 2 adult male patients whose preoperative imaging and fine needle cytology did not confirm the diagnosis of a parapharyngeal space mass and who completely resected the tumor through a combined cervical and oral approach. Finally, pathology confirmed ganglioneuroma; we also reviewed the English articles on parapharyngeal ganglioneuroma over the past 40 years, and summarized the diagnostic and treatment characteristics of parapharyngeal ganglioneuroma in combination with our cases to improve understanding of the disease.

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

Jayavelu

Anderson

Low

et al. 2022

Egypt J Otolaryngol

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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.

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Paediatric parapharyngeal ganglioneuroma

Cited by 4 publications

References 6 publications

Cervical ganglioneuroma mimicking a carotid body tumour

Cervical ganglioneuroma mimicking a carotid body tumour

Parapharyngeal Space Ganglioneuroma: Clinical Experience and Review of the Literature

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

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