[P-35] A case report of giant posterior mediastinal ganglioneuroma in a 4-year-old girl
Tutik Nur Ayni1,2 and Afiati Aziz1,2
- Dr Hasan Sadikin Hospital, Bandung, Indonesia
- Padjadjaran University, Bandung, Indonesia
Background: Ganglioneuroma is a rare (one per million population), differentiated, benign neurogenic tumour which originates from the neuroepithelium along the sympathetic ganglia. Most ganglioneuromas are thought to develop de novo rather than by maturation of a preexisting neuroblastoma, and are usually found in the posterior mediastinum and retroperitoneum.
Case Presentation: A 4-year-old girl patient presented at the thoracic surgery outpatients department with shortness of breath and cough for the last year. Computerized axial tomography (CT) showed a semisolid mass in the right superoanteromedioposterior mediastinum as high as 5th – 6th thoracic vertebra suggestive of a bronchogenic cyst. Histopathological examination of the specimen obtained from thoracostomy revealed tumour tissue comprising of ganglion cells individually distributed in Schwannian stroma. Neuritic processes produced by ganglion cells were enveloped by the cytoplasm of Schwann cells, so that there were no microscopic foci of naked neuropiI without Schwannian coverage. Immunohistochemistry showed positive results for S100 in both ganglion cells and Schwannian stroma.
Discussion and Conclusion: A posterior mediastinal mass presents with many differential diagnoses. These include a nerve sheath tumour, neuroblastoma, ganglioneuroblastoma, gastroenteric cyst, etc. Mediastinal ganglioneuromas arise from neural crest cells. These tumours are mostly asymptomatic, but massive tumours can present symptoms related to locoregional compression like dyspnoea and obscures its original location. Ganglioneuroma can be made as a differential diagnosis for bronchogenic cyst when a semisolid mass is found on CT. Definitive diagnosis can be made by histological examination. Complete surgical resection is the best choice for a curative treatment for mediastinal ganglioneuroma, due to the excellent prognosis.