Clinical features and treatment of central nervous system embryonal tumor, not otherwise specified_West China Medical Journal

Authors：

LI Qiang ¹ , CHEN Ni ² , LIU Yanhui ¹ ,  JU Yan ¹

1. Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P. R. China;
2. Department of Pathology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P. R. China;

Corresponding author：

JU Yan, Email: 46773206@qq.com

Keywords：

Central nervous system embryonal tumor, not otherwise specified; Craniotomy; Radiotherapy; Chemotherapy

DOI：

10.7507/1002-0179.201802051

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ObjectiveTo review the clinical records of patients with central nervous system (CNS) embryonal tumors, not otherwise specified (NOS); and summarize their clinical features, diagnosis, and treatment.MethodWe reviewed the data of patients with intracranial tumors admitted to Department of Neurosurgery of West China Hospital, Sichuan University from January 2014 to December 2016, and retrospectively analyzed the clinical features, diagnosis, and treatment of seven patients with CNS embryonal tumors, NOS.ResultsThere were 4 males and 3 females, and the mean age was 25.4 years old. The tumor was located in cerebral hemisphere in 5 patients, and in third ventricle in 2. Clinical presentation included headache, nausea, and vomiting due to intracranial hypertension, and focal neurological signs. All patients underwent craniotomy for tumor resection and postoperative pathology confirmed CNS embryonal tumor, NOS. The patients were followed up for 6 months to 3 years, and 2 patients died during follow-up.ConclusionsCNS embryonal tumor, NOS is malignant intracranial lesion, and has been enlisted as a separate entity under classification of CNS embryonal tumors. It has its unique radiological features, including rare occurrence of perilesional edema, cystic changes, and clear demarcation. Through comprehensive treatment including surgical resection, chemotherapy, and radiation therapy, patients can enjoy prolonged survival and improved quality of life.

Citation： LI Qiang, CHEN Ni, LIU Yanhui, JU Yan. Clinical features and treatment of central nervous system embryonal tumor, not otherwise specified. West China Medical Journal, 2018, 33(6): 719-722. doi: 10.7507/1002-0179.201802051 Copy

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1. Louis DN, Perry A, Reifenberger G, et al. The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol, 2016, 131(6): 803-820.
2. 曹勇, 张懋植, 赵继宗, 等. 幕上原始神经外胚层肿瘤 6 例报告. 首都医科大学学报, 2006, 27(6): 816-819.
3. 靳松, 崔世民, 刘梅丽, 等. 幕上原始神经外胚层肿瘤的 CT、MRI 和病理分析. 临床放射学杂志, 2006, 25(8): 707-710.
4. 杨凤, 郑小勇, 马卫波, 等. 幕上中枢神经系统胚胎源性肿瘤 NOS 型的 MRI 分析. 影像诊断与介入放射学, 2016, 25(6): 476-481.
5. Erdem E, Zimmerman RA, Haselgrove JC, et al. Diffusion-weighted imaging and fluid attenuated inversion recovery imaging in the evaluation of primitive neuroectodermal tumors. Neuroradiology, 2001, 43(11): 927.
6. 中华医学会病理学分会脑神经病理学组. 2016 世界卫生组织中枢神经系统肿瘤分类第 4 版修订版胚胎性肿瘤部分介绍. 中华病理学杂志, 2017, 46(7): 449-452.
7. Serowka K, Chiu Y, Gonzalez I, et al. Central nervous system (CNS) tumors in the first six months of life: the childrens hospital Los Angeles experience, 1979-2005. Pediatr Hematol Oncol, 2010, 27(2): 90-102.
8. Mcbride SM, Daganzo SM, Banerjee A, et al. Radiation is an important component of multimodality therapy for pediatric non-pineal supratentorial primitive neuroectodermal tumors. Int J Radiat Oncol Biol Phys, 2008, 72(5): 1319-1323.
9. Hsiao IH, Chen CH, Lee HC, et al. Primary leptomeningeal PNETs mimicking TB meningitis: a case report and literature review. Turk Neurosurg, 2017, 27(1): 155-159.
10. Desai KI, Nadkarni TD, Goel A, et al. Primary Ewing’s sarcoma of the cranium. Neurosurgery, 2000, 46(1): 62-68.

West China Medical Journal

Clinical features and treatment of central nervous system embryonal tumor, not otherwise specified

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