Malignant peripheral nerve sheath tumor (MPNST) arising from an adrenal gland ganglioneuroma: A case report

Abstract

Ganglioneuroma is a benign tumor arising from the neural crest cells. This tumor is rare compared to other benign neural tumors such as schwannoma or neurofibroma. Most ganglioneuromas are diagnosed in young patients and are most o en located in the posterior mediastinum followed by the retroperitoneum. Ganglioneuromas are benign tumors rarely di erentiate into malignant, and di erentiation into malignant peripheral nerve sheath tumors (MPNST) is extremely rare. To date, there are less than 20 reported cases of MPNST arinsing from ganglioneuroma in the literature We report a case of 24-year-old male with no history of childhood neuroblastoma or a familial neurofibromatosis, who presented with an adrenal gland mass discovered radiologically in the context of an exploration of low back pain. Our finding demonstrated that, although rare, spontaneous malignant transformation of ganglioneuroma into MPNST can occur.