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目的探讨幕上原始神经外胚瘤(supratentorial primitive neuroectodermal tumor,SPNET)的临床表现、影像和病理特征。方法对5例经病理证实的SPNET的资料,分析其临床表现、影像学组织病理学特征、治疗经过及预后。结果 5例中3例为儿童,2例为成人,主要临床表现为颅内高压症状。CT和MRI检查示肿瘤为幕上肿块,呈类圆形或分叶状,边界清楚,2例有钙化灶,3例无瘤周水肿。病理结果提示肿瘤组织主要由小圆形细胞组成,部分为梭形细胞,细胞密集成巢,由纤维结缔组织分隔成小叶状,核染色质颗粒状,核仁不明显,多数可见核分裂象,形成Homer Wright菊形团。结论 SPNET的影像学和组织病理学表现均有一定特征,影像学及临床表现相结合有助于术前与其他肿瘤相鉴别,提高诊断准确性。
Objective To investigate the clinical manifestations, imaging and pathological features of supratentorial primitive neuroectodermal tumor (SPNET). Methods The data of 5 cases of pathologically confirmed SPNET were analyzed, and their clinical manifestations, histopathological features, treatment course and prognosis were analyzed. Results Of the 5 cases, 3 were children and 2 were adults. The main clinical manifestations were intracranial hypertension. CT and MRI showed that the tumor was a supratentorial tumor with a round or lobulated shape, clear boundary, two cases of calcification and three cases of peritumoral edema. Pathological findings suggest that the tumor mainly composed of small round cells, some of the spindle cells, densely packed cells, separated by fibrous connective tissue into lobular, chromatin granular, not obvious nucleoli, the majority can be seen mitosis, the formation of Homer Wright daisy. Conclusion The imaging and histopathological findings of SPNET have certain characteristics. The combination of imaging and clinical manifestations can help to differentiate preoperatively from other tumors and improve diagnostic accuracy.