P3.CR-34 A Giant Ganglioneuroma of the Thoracic Spine

1/Case History: A 9-year old girl presented to our Department for a chronic cough. Her medical history revealed that she had pneumonia at the age of 2 years. The family history was marked by an allergic rhinitis in the father. On examination, she was afebrile.Full clinical examination revealed decreased breath sound in the left lung. 2/Investigations: Laboratory investigations were normal. The Chest CT-scan revealed a well-circumscribed mediastinal mass measuring 8.6cm*7.8cm*6.8cm. It extended through the neural foramen of T9 and also in the left chest cavity behind the aortic cross and the esophagus. Magnetic Resonance imaging showed a long intra-dural extra-medullary tumor extending from T6 to T11 levels. It extended through the left T9 neural foramen into the left thoracic cavity. The intervertebral foramen was enlarged. The thoracic aorta was totally sheathed. The lesion was mildly hypo-intense on T1-weighted imaging and heterogeneously hypo-intense on T2-weighted imaging. This radiological aspect could suggest a ganglioneuroma or a shwannoma. 3/Treatment: The multidisciplinary team decision was to perform surgery. Final histology demonstrated a well-circumscribed ganglioneuroma with negative margins. The case was reviewed at the Oncology multidisciplinary team. The decision was made to proceed with active follow-up without any adjuvant therapy given that it’s benign. The patient is currently doing well 2 years from her initial diagnosis. 4/Discussion: Thoracic intradural extra medullary Ganglioneuromas are very rare benign tumors of the autonomic nerve fibers. Most of them are incidentally discovered. In this case, our patient had no significant clinical symptoms apart from a chronic cough. The thoracic location, the potential of growth the large-size of the tumor (extending to the left chest wall with an intra-dural component) and the radiological profile, makes it therefore rare and unusual. It also illustrates the complexities of the management of this rare tumor. In fact, surgical treatment is usually safe and efficient. But, it can be more complicated in case of a such large-sized mass. So, a 2-step surgical intervention should be performed. Ganglioneuroma has commonly a good prognosis given its low-risk of recurrence and low-metastatic potential. However, it can become cancerous and spread to other organs.