All issues > Volume 44(12); 2001

Case Report

J Korean Pediatr Soc. 2001;44(12):1459-1462. Published online December 15, 2001.

A Case of Primitive Neuroectodermal Tumor of the Adrenal Gland

Sun Pil SP Jung¹, Chong Gwon CG Oh¹, In Seok IS Lim¹, Dong Keun DK Lee¹, Byoung Hoon BH Yoo¹

¹Department of Pediatrics, College of Medicine, Chungang University, Seoul, Korea

Abstract

Primitive neuroectodermal tumors(PNETs) are a group of small round cell tumors that may arise in the central or peripheral nervous system. In the extracranial location, these neoplasms may occur anywhere and at any age group, but most likely in the bone and soft tissue of children and young adults. The incidence of adrenal gland involvement is very rare. This tumor is diagnosed by histopathological, immunohistochemical, and molecular pathological examinations. PNETs are highly aggressive tumors, locally recurrent and rapidly metastasizing to lung, liver, lymph node, and brain. The outcome is poor with a 5 year survival rate below 40% in spite of aggressive combined treatment including surgery, chemotherapy, and radiation therapy. We experienced a case of PNET of adrenal gland, mimicking ruptured Wilms tumor on the US and CT scan.

Keywords :Primitive neuroectodermal tumors, Adrenal gland