Ganglioneuroma in Head and Neck: A Case Report of a Laryngeal Ganglioneuroma and a Systematic Review of the Literature

Abstract

Introduction: Ganglioneuroma (GN) is a rare, benign tumor of the autonomic nervous system. It is seldom located in the head and neck (HN) region. GN typically presents as a slow-growing, painless mass, often leading to delayed diagnosis. Case report: We report a unique case of laryngeal-originating GN in a 43-year-old female who presented with worsening dysphonia and dyspnea. Imaging revealed a large mass originating from the larynx. Histological examination confirmed the diagnosis. The tumor was surgically excised with preservation of key structures in the neck. At the follow-up, the patient experienced a significant improvement in symptoms. Material and methods: A systematic literature review following PRISMA guidelines was conducted in January 2024 to investigate the common sites of GN in the HN region and the complications associated with its treatment. Results: In a total of 58 articles, we studied 65 patients, mostly under 30 years old. Surgical excision remains the primary treatment, and post-operative complications were mostly neurological. Discussion: GNs are generally slow-growing and asymptomatic, but they can reveal themselves when compressing nearby structures, especially in the HN region. In symptomatic cases or when the tumor exhibits significant growth or hormonal activity, surgical resection is required. The lateral cervical approach is the most common one. The risk of postoperative complications and recurrence underscores the need for careful surgical planning and long-term follow-up. Conclusion: This unique laryngeal GN case highlights the importance of considering GN in the differential diagnosis of HN masses. Further large-scale studies are warranted to establish evidence-based protocols for their management, especially in the HN region.

Keywords: cervical ganglioneuroma; ganglioneuroma management; head and neck; laryngeal tumors; neuroblastic tumors.

Figure 1

Preoperative fibrolaryngoscopy.

Figure 2

Axial (A,B), coronal (C,D), and sagittal (E) contrast-enhanced CT scan of the neck.

Figure 3

Histological findings of laryngeal ganglioneuroma. (a) Hematoxylin and eosin ×25; (b) hematoxylin and eosin ×200; (c) synaptophysin stain.

Figure 4

PRISMA 2020 flow diagram for new systematic reviews.

Figure 5

Traffic lights plot [2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43,44,45,46,47,48,49,50,51,52,53,54,55,56,57].

Figure 6

Weighted bar plot.