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目的探讨成人幕上原始神经外胚叶肿瘤(AS-PNET)的临床病理学特征及鉴别诊断要点。方法收集8例AS-PNET的临床及影像学资料,观察其病理学形态及免疫表型,并复习相关文献,探讨该肿瘤的病理诊断和鉴别诊断要点。结果患者男性3例,女性5例,发生部位以颞叶、额叶、顶叶为主。临床表现主要为颅内压增高、头痛、恶心呕吐,意识障碍。CT表现为等密度或高密度影,增强后可强化,肿瘤内可见钙化或微出血。MRI显示肿块界限不清楚或清楚,异质信号,增强后不同程度强化,瘤周出现不同程度水肿。镜下肿瘤组织与周围脑组织边界不清。瘤细胞主要由未分化的小细胞构成,细胞圆形、短梭形,胞质少,核浆比高,核深染,核仁不明显,核分裂象易见。1例可见瘤巨细胞,微血管增生明显,肾小球样血管;2例黏液背景下部分区域可见微囊结构形成,局部可见菊形团;2例局部区域出现少突细胞瘤样分化;2例局部区域出现胶质分化。免疫组化:CD56、Syn、β-tublin、MAP-2、INI-1、Fli-1(4/8)和Olig-2(2/8)(+),CD99(3/8)弱(+),nestin(7/8)部分细胞(+),Neu N(1/8)局灶(+);胶质分化区域GFAP(2/8)(+),未分化小细胞区域(-);Cg A、EMA、C-myc(-);p53(4/8)核弥漫(+),Ki-67阳性指数30%~80%。结论 AS-PNET罕见,其确诊需结合临床、影像学、组织病理学及免疫组化等进行综合分析。
Objective To investigate the clinicopathologic features and differential diagnosis of adult supratentorial primitive neuroectodermal tumors (AS-PNET). Methods The clinical and imaging data of 8 cases of AS-PNET were collected and the pathological morphology and immunophenotype were observed. The related literatures were reviewed and the main points of pathological diagnosis and differential diagnosis of AS-PNET were reviewed. Results There were 3 males and 5 females, with the temporal lobe, frontal lobe and parietal lobe as their main sites. Clinical manifestations of increased intracranial pressure, headache, nausea, vomiting, disturbance of consciousness. CT showed isodense or high-density, enhancement can be enhanced, the tumor visible calcification or micro-bleeding. MRI showed unclear tumor mass or clear, heterogeneous signal enhancement enhanced to varying degrees, the tumor showed varying degrees of edema. Microscopic tumor tissue and surrounding brain tissue is unclear. Tumor cells are mainly composed of undifferentiated small cells, round cells, short fusiform, less cytoplasm, high ratio of nuclear plasma, nuclear stained, nucleoli are not obvious, mitotic easy to see. 1 case of tumor giant cells, microvascular hyperplasia, glomerular vascularization; 2 cases of mucus in the context of the formation of microcirculation can be seen in some areas, partially visible chrysanthemum; 2 cases of local areas of tumorous differentiation of oligodendrocytes; 2 cases Local areas appear glial differentiation. Immunohistochemistry: CD56, Syn, β-tublin, MAP-2, INI-1, Fli- 1 (4/8) and Olig- 2 (2/8) ), nestin (7/8) cells (+), Neu N (1/8) focal (+), glial differentiation area GFAP (2/8) Cg A, EMA, C-myc (-); p53 (4/8) nuclear diffuse (+), Ki-67 positive index of 30% to 80%. Conclusion AS-PNET is rare and its diagnosis should be combined with clinical, imaging, histopathology and immunohistochemistry.