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原发性脊髓硬膜内髓外尤因肉瘤/外周原始神经外胚层肿瘤临床伪装成神经源性肿瘤:一例报告及文献复习

Primary Spinal Intradural Extramedullary Ewing's Sarcoma/Peripheral Neuroectodermal Tumour Masquerading Clinically as a Neurogenic Tumour: A Case Report and Review of Literature.

作者信息

Praveen B K, Das Saikat, Gupta Manish, Joshi Deepti, Panwar Hemlata

机构信息

Department of Pathology and Lab Medicine, All India Institute of Medical Science Bhopal, Saket Nagar, Habib Ganj, Bhopal, Madya Pradesh, India.

Department of Radiotherapy, All India Institute of Medical Science Bhopal, Saket Nagar, Habib Ganj, Bhopal, Madya Pradesh, India.

出版信息

Ann Neurosci. 2023 Oct;30(4):251-255. doi: 10.1177/09727531231190979. Epub 2023 Sep 14.

Abstract

Ewing's Sarcomas (ES)/Peripheral neuroectodermal tumour (pPNET) are heterogenous group of rare, highly malignant, undifferentiated primitive round-cell neoplasms of neuroectodermal origin. pPNETs are seldom observed to involve the spine of which Spinal Intradural Extramedullary Extraosseous Primary ES/pPNET are extremely rare. We report a case of a 23-year-old male with complaints of low backache and hip pain radiating to the left inguinal region for four months. Radiology findings were suggestive of a neurogenic tumour. Cytomorphology, histomorphology and immunohistochemistry evaluation were done. Diagnosis was consistent with ES/pPNET. Careful correlation between clinical history, cytomorphology, histopathology, immunohistochemical and molecular analysis can help to distinguish primary spinal ES/PNET from other primary spinal tumours and will help clinicians to start treatment at the earliest.

摘要

尤因肉瘤(ES)/外周原始神经外胚层肿瘤(pPNET)是一组异质性的罕见、高度恶性、未分化的神经外胚层起源的原始圆形细胞肿瘤。很少观察到pPNET累及脊柱,其中脊髓硬膜内髓外骨外原发性ES/pPNET极为罕见。我们报告一例23岁男性,主诉下背痛和臀部疼痛放射至左腹股沟区4个月。影像学检查结果提示为神经源性肿瘤。进行了细胞形态学、组织形态学和免疫组化评估。诊断与ES/pPNET一致。临床病史、细胞形态学、组织病理学、免疫组化和分子分析之间的仔细关联有助于将原发性脊柱ES/PNET与其他原发性脊柱肿瘤区分开来,并将帮助临床医生尽早开始治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/995f/10662269/8e5701046370/10.1177_09727531231190979-fig1.jpg

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