INTRODUCTION: Ganglioneuroma is a rare posterior mediastinal tumor which usually presents during childhood. It is a well differentiated benign tumor composed of mature ganglion cells. Ganglioneuroma is usually asymptomatic and when symptoms present they are secondary to mass effect from tumor extension. We describe an adult patient with thoracic ganglioneuroma who presented with chest tightness and shortness of breath. We also illustrate the clinical and histopathological features of a giant thoracic ganglioneuroma.
CASE PRESENTATION: A 27 year-old African American female with no past medical history presented with chest tightness and dyspnea on exertion for 2 months. She was hemodynamically stable and oxygen saturation was 99% on room air without signs of acute respiratory distress. Auscultation and percussion of the chest revealed decreased breath sounds and dullness on the right. Complete Blood Count, Basic metabolic panel and arterial blood gases were within normal limits. Chest x-ray revealed soft tissue mass density on the right mid and lower lung with a 25% right-sided pneumothorax. A CT scan of the chest revealed a soft tissue mass occupying two thirds of the right hemithorax adjacent to the pleura and the ribs. MRI of the thoracic spine confirmed a large right paraspinal tumor extending from T3 to T12 with extension into the neural foramina at multiple levels. Barium esophagogram showed displacement of the distal esophagus to the left with no esophageal involvement. Patient was evaluated by Pulmonary, Thoracic surgery andNeurosurgery services and underwent bronchoscopy, hemilaminectomy of T4-T7, right thoracotomy and resection of the giant pleural tumor. Histopathological examination revealed a ganglioneuroma invading the right pleura. Patient's symptoms resolved and she is doing very well.
DISCUSSIONS: The differential diagnosis of posterior medistinal mass includes neurogenic tumors. Ganglioneuroma is a neurogenic tumor that arises from sympathetic ganglion cells. The incidence of ganglioneuroma is unknown. The tumor is generally encapsulated and attached to a sympathic or intercostal nerve trunk. It may present with chest pain secondary to thoracic bone compression or erosion of the nerves, cough, dyspnea or hemoptysis. These symptoms are related to intrathoracic extension of the tumor. Intraspinal growth with intrathoracic extension is a rare complication of ganglioneuroma and should be approach by neurosurgical and thoracic procedures. Ct scan and MRI are useful to localize the tumor and define the extension. Complete resection is generally curative and local recurrence is uncommon.
CONCLUSION: Although it is rare, ganglioneuroma should be included in the differential diagnosis of posterior mediastinal and intrathoracic masses in young adults. Multidisciplinary team approach is necessary for the proper diagnosis and treatment of intrathoracic ganglioneuroma.
DISCLOSURE: Juan Rojas Balcazar, No Financial Disclosure Information; No Product/Research Disclosure Information