Contributor: Gordon K. Klintworth
Ganglioneuroma has been documented in numerous sites, including the gastrointestinal tract and sympathetic chain (in retroperitoneum, and posterior mediastinum). The tumor is extremely rare in the orbit and may be an extension from a paranasal sinus. The neoplasm is circumscribed and its cut surface is tan or gray with a whorled appearance. Ganglioneuroma may be found in association with neurofibromatosis type I and multiple endocrine neoplasia syndrome. Despite being benign ganglioneuromas have been found in persons with metastatic neuroblastoma [neuroblastoma - metastatic]. In such situations the ganglioneuroma has been thought to represent a differentiation from rests of neuroblastoma cells.