A Rare Cause of Dysphagia: A Giant Ganglioneuroma in Parapharyngeal Space

Abstract

Parafarengeal ganglioneuroma is a very rare benign tumor originating from the sympathetic nervous system that produces mass and functional effects. Ganglioneuroma is most commonly caused by the posterior mediastinal, retroperitoneal area and adrenal glands. Generally, they do not present any additional signs or symptoms other than mass. Horner’s syndrome may sometimes occur due to the compression of the sympathetic chain. A 41-year-old male patient with a long-standing mass in the lateral compartment of the left neck was admitted to our clinic. The patient was operated, and the pathological result of the mass was reported as ganglioneuroma. We present a rare case of ganglioneuroma in paraphryngeal space by reviewing the current literature.

Introduction

Ganglioneuroma is the most distinctive benign counterpart of neuroblastoma and originates from neural crest cells that normally migrate to the adrenal medulla and sympathetic ganglia [1–3]. Ganglioneuromas can occur wherever there is a sympathetic chain. They are most commonly found in the posterior mediastinum, retroperitoneum and adrenal medulla [1, 2, 4–6]. The incidence in the neck is between 1–5 and 8% in some publications [1, 2, 4–8]. They typically do not constitute symptoms. They are rarely symptomatic depending on the compression effect of the mass [1, 2, 4–8]. Symptoms related to hormone secretion in ganglioneuromas of the neck have not been reported or are very rare [5, 9]. The treatment is surgical.

Case Report

A 41-year-old male was admitted with a left-lateral neck mass slow growing over a 3 years duration. The patient had no comorbid disease. The tumor was painless, firm, solid, semi-mobile, non-fluctuant and was not associated with any systemic or compression-related symptoms. No symptoms of Horner's syndrome, including ptosis, myosis, ipsilateral facial anhidrosis and flushing, were observed. The mass extended from the left parapharyngeal region to the third part of the neck. It was measured 10 × 5 cm in size, and the overlying skin was normal. Routine laboratory tests were within normal limits. Ultrasonography showed a mass located 2,3th, region in the left posterior neck which displayed solid, hypoechoic and well-encapsulated characteristics. The magnetic resonance imaging (MRI) identified ovoid mass located in the left posterior lateral neck from 2 to 3 levels and measured 93 × 35.5 × 45 mm (Fig. 1). MRI showed low-signal intensity on T1WI. T2WI showed higher signal intensity.

Fig. 1.

MRI performed before surgical resection a T1 weighted non-contrast sections, b T2 weighted contrast sections

Transoral, transcervical, transparotid and/or combined approaches are preferred in the excision of masses extending to the parapharyngeal area. The transcervical approach was preferred due to the extension of the mass from the parapharyngeal area to the third part of the neck. Complete surgical excision of the mass was performed under general anesthesia through a transcervical approach. All cranial nerves and structures including the accessory nerve were preserved during surgery. The carotid artery and internal jugular vein were preserved. The excised mass was encapsulated, solid and ovoid white-yellowish(Fig. 2). The mass was measured 10 × 5 × 4 cm in size(Fig. 2). Histopathologic examination revealed a Schwannian spindle cell proliferation with wavy nuclei and occasional mature ganglion cells with large, granular eosinophilic cytoplasm, single round nucleus and prominent nucleolus (Fig. 3). Pathologically it was reported as neuroganglioma. The patient was operated and discharged. In the recent follow-up of the patient, myosis developed in the left eye. However, in the follow-up of the patient, myosis returned to normal.

Fig. 2.

Macroscopic appearance of the tumor

Fig. 3.

a Intersecting fascicles of mature Schwannian stromal component, H&E, × 100, b a mature ganglion cell containing granular, yellow–brown cytoplasmic pigment, H&E, × 400

Discussion

Peripheral neuroblastic tumors are divided into three subgroups depending on the degree and type of neuroblastic differentiation and the degree of development of Schwannian stroma: neuroblastomas, ganglioneuroblastoma and ganglioneuromas [7]. They all originate from neural crest cells and are considered different stages of maturation. Maturation is almost non-existent in neuroblastoma and is highest in ganglioneuromas. Ganglioneuromas represent the most mature form of mature ganglion cells scattered in a dominant Schwannian stroma. In the meantime, ganglioneuromas are included no atypia, mitosis or necrosis. Therefore is considered benign as compared to the other two forms. It is accepted that ganglioneurons do not metastasis due to their histopathological features [2, 8].

Ganglioneuromas can occur wherever there is a sympathetic chain. However, when the literature is reviewed, it occurs most commonly in the posterior mediastinum, retroperitoneum and adrenal glands [1–6, 10]. However, when the literature is reviewed, the rates for the neck are between 1 and 5%[1, 2, 4–6, 8]. Patients with cervical sympathetic chain involvement rarely have involvement in the parapharyngeal space, hypoglossal nerve, intervertebral foramen and spinal canal [1, 4]. It is more common in women [1, 4, 5].

Ganglioneuromas usually do not show signs or symptoms other than mass. Sometimes compression symptoms may be seen depending on the anatomic location of the mass [1]. According to the area where the tumor develops, dysphagia, dysarthria, horner’s syndrome, hearing loss and pain may occur [1, 2, 4–9].

The diagnosis of ganglioneuroma is made by histopathological diagnosis. Fine-needle cytology of preoperative mass is not generally insufficient. Computerized tomography(CT)and magnetic resonance imaging (MRI) provide valuable information about the size, location, composition of the mass and its relationship to adjacent major structures that are of great help in determining the surgical plan [1, 4–9].

The treatment of ganglioneuromas is surgery [1, 2, 4–9]. The most appropriate surgical technique should be chosen according to the location of the lesion. When the literature is reviewed, we see that transoral or many external surgical neck approaches are applied according to the localization of the mass. Complete removal of the mass is usually curative. However, the removal of the mass with subtotal or partial approaches to prevent morbidity has also yielded satisfactory results. Radiation therapy is not recommended as it leads to morbidity and secondary malignancy in the treatment of ganglioneuromas [9, 10]. The prognosis of ganglioneuromas which has by surgical treatment is very good.