Ganglioneuroma

Ganglioneuroma

Defi nition Ganglioneuromas (GNs) are benign di ff erentiated tumours of neural crest-derived cells in the autonomic nerves. Incidence GNs are rare, a ff ecting one per million of the population. Most are sporadic but they can be associated with neurofibromatosis type II and MEN /uni00A0 2B. Pathology GNs are benign and are most often located in the mediastinum (20%), retroperitoneum (10%) and adrenal gland (30%). However, GNs can arise anywhere sympathetic nervous tissue is found, such as tongue, bladder, uterus, bone and skin. They are composed of an admixture of ganglion cells and Schwannian stroma/cells. Clinical presentation GNs develop in childhood but typically present later as they are non-secreting and slow growing. Two-thirds of patients are under the age of 20 years. They are usually asymptomatic Theodore Schwann , 1810–1882, Professor of Anatomy , Leuven, Belgium. Michel Gagner , b. 1960, Professor of Surgery , Montreal, Canada. Martin K Walz , contemporary , Professor of Surgery , Essen, Germany . and are identified incidentally . When large, GNs may cause local pressure symptoms such as abdominal pain or bloating. Occasional reports of elevated metanephrines exist and the di ff erential diagnosis is PCC. Diagnosis Once a mass is discovered CT and MRI are usually performed to characterise the lesion. They are well defined with a capsule and calcification may be present. GN has a low T1- and a high T2-weighted signal on MRI. Radiology cannot defini - tively diagnose a GN; histology is required and so preoperative diagnosis can be challenging. Treatment Excision is the treatment of choice. A laparoscopic approach is preferred. The prognosis is excellent as recurrences are rare.