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Ganglioneuromas involving the hypoglossal nerve and the vagus nerve in a child: Surgical difficulties.

Introduction

Ganglioneuromas are tumors of the sympathetic nervous system that arise from the Schwann cells of the peripheral nervous system. They are among a trio of neurogenic tumors that also includes neuroblastomas and ganglioneuroblastomas. Ganglioneuromas represent the benign and well-differentiated form, neuroblastomas represent the malignant and undifferentiated form, and ganglioneuroblastomas represent an intermediate form.

Ganglioneuromas can found anywhere along the sympathetic nerve chain. They are most often seen in the posterior mediastinum and the paraspinal retroperitoneum. (1) In rare cases, they have been seen in the urinary bladder, bowel wall, and gallbladder. (2) In the head and neck, they usually arise from the cervical sympathetic chain. Other rare sites include the hypoglossal nerve, the vagus nerve, (3) the pharynx, and the larynx. They can also arise from the intervertebral foramen and extend into the spinal cord. Ganglioneuromas are most common in young adults (mean age: 20.6 yr), and they have a slight predilection for females. (4)

Parapharyngeal involvement of a ganglioneuroma is extremely rare. In this article, we describe a case of multiple ganglioneuromas of the parapharyngeal space that had arisen from two different cranial nerves. To the best of our knowledge, no such case has been previously reported in the literature. We also discuss the difficulties we faced during surgical excision.

Case report

A 10-year-old girl presented to our outpatient facility with a 2-year history of a painless and slowly progressive swelling in the left side of her neck. She also reported a 1-year history of hoarseness. She experienced no difficulty in opening her mouth, and she reported no nasal bleeding, nasal obstruction, or nasal regurgitation. She had no history of relevant trauma or surgery.

On examination, a firm 6 x 4-cm swelling was palpated in the left submandibular area (figure 1). The swelling was not mobile, pulsatile, tender, transluminant, or attached to the skin. Inspection of the oral cavity revealed a left hypoglossal palsy.

Contrast-enhanced computed tomography (CT) demonstrated multiple well-defined, heterogeneously enhancing swellings in the left parapharyngeal space (figure 2). These swellings had resulted in an inward bulging of the oropharynx.

Intraoperatively, two tumors were seen in the left parapharyngeal space; both had a bosselated surface. One tumor involved the hypoglossal nerve (figure 3) and the other, deeper tumor involved the vagus nerve. Neither tumor could be separated from its attached nerve, so excision necessitated that both nerves be sacrificed. On gross examination, both tumors measured approximately 4 x 5 cm (figure 4).

Postoperatively, the girl was extubated uneventfully. During the immediate postoperative period, she complained of aspiration of fluids, which was managed with positional maneuvers and exercises for the compensatory adduction of the vocal folds. Postoperative histopathology identified both tumors as ganglioneuromas (figure 5).

During 1 year of follow-up, the patient experienced no recurrences. Although she still exhibited hypoglossal nerve and vocal fold palsy, she was asymptomatic.

Discussion

In the head and neck, ganglioneuromas can appear as an asymptomatic mass or in association with noticeable nerve palsies. Our patient presented with glossal atrophy and a deviation of the tongue secondary to her tumor's involvement of the hypoglossal nerve. Even though the vagus nerve was found to be involved intraoperatively, she had no vocal fold palsy at the time of presentation.

Histopathologically, ganglioneuromas are made up of ganglion cells of varying maturity and stroma. Neuroblasts are the immature undifferentiated counterparts of ganglion cells. These neuroblasts are surrounded by the supporting stromal cells. Other features include necrosis, mitosis, hemorrhage, fibrosis, calcification, and lymphocytic infiltration. The presence of neuroblasts indicates a malignancy (neuroblastoma). Both ganglioneuromas and neuroblastomas are covered with a pseudocapsule.

Based on histopathologic findings, the prognosis can be classified by either the Pediatric Oncology Group (POG) system or the Schimda system. The POG system classifies tumors according to the degree of differentiation of the ganglion cells. The Schmida system adds to the assessment other histologic features such as stromal components, tumor grade, and nuclear morphology.

Operating in the parapharyngeal space has always been a challenge for head and neck surgeons because it is close to so many vital structures. Moreover, since this space has the potential to expand, tumors often reach a substantial size before they become clinically detectable.

The key to performing an uneventful surgery in the parapharyngeal space is adequate exposure. Different methods have been adopted to increase exposure and improve surgical access. For example, when performing surgery via a lateral approach, it is necessary to identify and delineate the carotid vessels and the internal jugular vein. The tumor must then be dissected off the surrounding structures. Efforts should be made to dissect off any nerve that is attached to the tumor. However, as in our case, some tumors are inseparably attached to the nerve, and therefore the nerve must be cut and grafted with a cable graft. If a tumor is large and involves the medial parapharyngeal space, the stylomandibular ligament must be cut to expose the tumor's more superior and medial aspects.

Our patient's hypoglossal tumor had extended superiorly to the transverse process of the second cervical vertebrae, and the vagal tumor was intimately related to the internal carotid artery. The internal jugular vein, which was already compressed and tethered to the tumor, had to be ligated.

Both the hypoglossal nerve tumor and the vagus nerve tumor had to be sacrificed during resection. The stylomandibular ligament had to be ligated to facilitate the removal of the vagus nerve tumor, which had extended medially and caused an inward bulge in the oropharynx. If further exposure had been required, we could have performed a lateral mandibulotomy, but we did not perform such a procedure in our young patient because it would have caused a dental malocclusion in the future and because the benign nature of the tumor did not warrant it.

To the best of our knowledge, only 3 other cases of multiple ganglioneuromas have been reported in the Englishlanguage literature. (4) In these cases, both tumors arose from the sympathetic ganglia. We present what we believe is the first case of multiple ganglioneuromas that arose from different cranial nerves. Also, ours is only the third case in which the hypoglossal nerve was involved. (5)

References

(1.) Albonico G, Pellegrino G, Maisano M, Kardon DE. Ganglioneuroma of parapharyngeal region. Arch Pathol Lab Med 2001; 125 (9): 1217-18.

(2.) Danosos DA, Santos VB, Ruffy ML,et al. Ganglioneuroma of the parapharyngeal space. Bull N Y Acad Med 1980; 56 (7): 616-21.

(3.) Clay RC. Ganglioneuroma of the nodose ganglion of the vagus. Ann Surg 1950; 132 (1): 147-52.

(4.) Ma J, Liang L, Liu H. Multiple cervical ganglioneuroma: A case report and review of the literature. Oncol Lett 2012; 4 (3): 509-12.

(5.) Hallur N, Sikkerimath BC, Gudi SS,et al. Parapharyngeal ganglioneuroma of hypoglossal nerve in a 4 year old girl: A rare case report. J Maxillofac Oral Surg 2012; 11 (3): 343-6.

From the Department of Otolaryngology-Head and Neck Surgery (Dr. Bakshi) and the Department of Histopathology (Dr. Nada), Post Graduate Institute of Medical Education and Research, Chandigarh, India; the Department of ENT, King Faisal University College of Medicine, Al-Ahsa, Kingdom of Saudi Arabia (Dr. Mohammed); and the Vijaya E.N.T. Care Centre, Bangalore, India (Dr. Lele).

Corresponding author: Dr. Abdul Wadood Mohammed, Valiyatharayil House, (PO) Edakkazhiyur, (DT) Thrissur, Kerala, India 680515. Email: wadood_abd@yahoo.co.in

February 24, 2016 by Jaimanti Bakshi, MS, MNAMS; Abdul Wadood Mohammed, MS; Saudamini Lele, MS; Ritambra Nada, MD

Caption: Figure 1. The left submandibular swelling is seen at presentation.

Caption: Figure 2. CT shows the multiple swellings in the left parapharyngeal space.

Caption: Figure 3. Intraoperative photo shows the tumor attached to the hypoglossal nerve.

Caption: Figure 4. Both tumors have a bosselated surface.

Caption: Figure 5. Histopathology shows spindle cells and a few large ganglion cells.
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Author:Bakshi, Jaimanti; Mohammed, Abdul Wadood; Lele, Saudamini; Nada, Ritambra
Publication:Ear, Nose and Throat Journal
Date:Feb 1, 2016
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