成人鞍上原始神经外胚层肿瘤

Suprasellar Primitive Neuroectodermal Tumor in an Adult.

作者信息

Espino Barros Palau Angelina, Khan Khurrum, Morgan Michael L, Powell Suzanne Z, Lee Andrew G

机构信息

Department of Ophthalmology (AEBP), Centro Médico Zambrano Hellion-Tec Salud, Monterrey, Mexico; Department of Ophthalmology (KK, AGL), Baylor College of Medicine, Houston, Texas; Department of Ophthalmology, Houston Methodist Hospital, Houston, Texas; Department of Pathology and Genomic Medicine (MLM, AGL), Houston Methodist Hospital, Houston, Texas; Departments of Ophthalmology (AGL), Neurology, and Neurosurgery, Weill Cornell Medical College, Houston, Texas; Department of Ophthalmology (AGL), UTMB Galveston, Texas; The UT M.D. Anderson Cancer Center (AGL), Houston, Texas; and The University of Iowa Hospitals and Clinics (AGL), Iowa City, Iowa.

出版信息

J Neuroophthalmol. 2016 Sep;36(3):299-303. doi: 10.1097/WNO.0000000000000312.

DOI:10.1097/WNO.0000000000000312

PMID:26517622

Abstract

Primitive neuroectodermal tumors (PNET) of the central nervous system (CNS) are a heterogeneous group of embryonal malignancies that are composed of undifferentiated or poorly differentiated neuroepithelial cells. Supratentorial PNET is the second most common CNS embryonal malignancy in children, but it is rare in adults. We report the case of a 31-year-old woman with bilateral vision loss and a bitemporal hemianopia. Neuroimaging revealed a suprasellar mass, and pathology was consistent with PNET. After surgical debulking of the tumor followed by radiation therapy and chemotherapy, the patient had significant visual recovery and remained stable over 14 months of follow-up.

摘要

中枢神经系统（CNS）的原始神经外胚层肿瘤（PNET）是一组异质性的胚胎性恶性肿瘤，由未分化或低分化的神经上皮细胞组成。幕上PNET是儿童中第二常见的中枢神经系统胚胎性恶性肿瘤，但在成人中罕见。我们报告一例31岁女性，有双侧视力丧失和双颞侧偏盲。神经影像学检查发现鞍上有一肿块，病理结果与PNET相符。在对肿瘤进行手术减瘤，随后进行放疗和化疗后，患者视力有显著恢复，在14个月的随访期间病情保持稳定。