Journal of the Korean Pediatric Society 2001;44(12):1459-1462.
Published online December 15, 2001.
A Case of Primitive Neuroectodermal Tumor of the Adrenal Gland
Sun Pil Jung, Chong Gwon Oh, In Seok Lim, Dong Keun Lee, Byoung Hoon Yoo
Department of Pediatrics, College of Medicine, Chungang University, Seoul, Korea
부신에서 발생한 원시 신경 외배엽 종양 1례
정순필, 오종권, 임인석, 이동근, 유병훈
중앙대학교 의과대학 소아과학교실
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.
Key Words: Primitive neuroectodermal tumors, Adrenal gland


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