Ganglioneuroma of the Sacral Spine: A Case Report and Literature Review

 Spinal ganglioneuromas are benign tumors that often present symptoms related to compression of nerve elements. They are generally rare. Treatment is essentially surgical, generally improves symptoms and is accompanied by a low probability of tumor recurrence. We describe the case of a 49-year-old man with a ganglioneuroma involving the right third sacral nerve root. Revealed by a cauda equina syndrome. Treatment consisted of complete excision of the tumor. The outcome was favorable, with slight recovery of genito-phincter disorders after 6 months.

Introduction

Ganglioneuromas are benign tumours that originate in tissue derived from the neural crest and are therefore closely linked to the embryonic development of the sympathetic nervous system [1], [2]. However, ganglioneuromas arising in the nerve root are extremely rare [3].

Case Report

This is a 49-year-old male patient with no previous pathological history, admitted to our department for chevale tail syndrome with genital-sexual disturbances. Clinical examination revealed saddle anaesthesia with no sensory or motor deficit. A perineal electromyogram was performed, suggesting a ponytail syndrome from S1 to S5. Magnetic resonance imaging revealed a lesion dependent on the nerve roots of S3 (Fig. 1). The patient underwent complete excision of the lesion, and pathological examination revealed the presence of spindle cells consistent with a neuronal ganglion origin. The tumor was diagnosed as a ganglioneuroma. Postoperatively the patient reported a slight recovery of genito- sphincter disorders.

Fig. 1. Lumbar MRI sagittal section showing a rounded lesion opposite S3.

Discussion

Ganglioneuroma is a benign, neurogenic tumor. It is a rare condition, with an estimated frequency of seven per million individuals [4]. They arise from neural crest cells and can affect any part of the sympathetic system. It is composed of mature ganglion cells and a stroma containing nerve cells and a schwanian contingent [5].

The retroperitoneum is the most common site for these tumors, followed by the mediastinum; sacral sites are exceptional. They occur at all ages but are particularly common in children and young adults [6]. The clinical signs and symptoms of spinal ganglioneuromas vary according to their location in the spinal column and may sometimes be discovered by chance. Radiological diagnosis of these tumors is difficult. However, imaging allows us to pinpoint the location of the tumour and its relationship with neighbouring organs [7].

The definitive diagnosis of ganglioneuroma is anatomopathological. The treatment of choice is surgical resection [8]. Long-term prognosis is excellent, whatever the tumour location, so regular radiological follow-up with neurological examination and radiological assessment is necessary, even after complete excision.

Conclusion

Despite its rarity and benign nature, ganglioneuroma deserves to be recognized. Diagnosis is often delayed. Imaging, in particular CT and MRI, confirms the tumor’s location and relationship, and predicts its resectability.