J Korean Pediatr Soc.  2001 Dec;44(12):1459-1462.

A Case of Primitive Neuroectodermal Tumor of the Adrenal Gland

• Jung SP
• Oh CG
• Lim IS
• Lee DK
• Yoo BH

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.

Keyword

Primitive neuroectodermal tumors; Adrenal gland

MeSH Terms

Adrenal Glands*
Brain
Child
Drug Therapy
Humans
Incidence
Liver
Lung
Lymph Nodes
Neural Plate
Neuroectodermal Tumors, Primitive*
Peripheral Nervous System
Survival Rate
Tomography, X-Ray Computed
Wilms Tumor
Young Adult