论文部分内容阅读
目的探讨多形性黄色瘤型星形细胞瘤(PXA)的临床病理特征、诊断及鉴别诊断、治疗及预后。方法对南京军区南京总医院1980-2004年间6287例中枢神经系统肿瘤中的15例PXA(0．2％),以及2例会诊病例,进行临床病理学观察,免疫组织化学SP法检测8种抗体的表达:胶质纤维酸性蛋白、波形蛋白、S-100、上皮细胞膜抗原、突触素、神经微丝、CD68及CD34,获得其中10例的随访资料。结果患者年龄12—55岁,平均30．8岁,男6例,女11例。主要症状为癫痫发作、头痛、头晕等。肿瘤发生于幕上者16例,占94．1％,其中发生于颞叶者7例,占41．2％。肿瘤大小2～7 cm,平均4．3 cm,9例有囊性变。除2例会诊病例外,全切除12例,次全切除3例。随访10例,生存8例,生存时间10个月～13年7个月,平均生存6年,生存10年以上者2例。组织学特征为:单核或多核巨怪瘤细胞、梭形细胞和泡沫样瘤细胞混合而成,肿瘤中有丰富的网状纤维及淋巴细胞浸润,缺乏坏死,核分裂象无或少。胶质纤维酸性蛋白、波形蛋白及S-100蛋白免疫组织化学染色均呈弥散阳性表达,CD34阳性率为77％。1例伴有间变特征的PXA,有较多核分裂象(≥5个／10 HPF)。2例有脑实质及血管周围间隙的浸润。1例影像学检查提示肿瘤复发及脑膜播散。结论PXA属WHOⅡ级肿瘤,肿瘤全切除及组织学为典型性PXA者预后较好,少数PXA可复发及问变。瘤细胞巨大、怪异,容易误诊为WHOⅣ级的巨细胞胶质母细胞瘤,两者的鉴别要点在于PXA部分可见泡沫样瘤细胞,核分裂象无或少,缺乏坏死。瘤细胞CD34的阳性表达有助于PXA的诊断。
Objective To investigate the clinicopathological features, diagnosis, differential diagnosis, treatment and prognosis of pleomorphic xanthooblastoma (PXA). Methods Fifty-two cases of PXA (0.2%) in 6287 CNS tumors from Nanjing General Hospital of Nanjing Military Region from 1980 to 2004 were enrolled in this study. Clinical pathology was also observed in 2 cases. 8 kinds of antibodies were detected by immunohistochemical SP method The glial fibrillary acidic protein, vimentin, S-100, epithelial cell membrane antigen, synaptophysin, neurofilament, CD68 and CD34 were used to obtain the follow-up data of 10 cases. Results Patients aged 12-55 years, mean 30.8 years, 6 males and 11 females. The main symptoms of seizures, headache, dizziness and so on. Tumors occurred in the screen on 16 cases, accounting for 94.1%, of which occurred in the temporal lobe in 7 cases, accounting for 41.2%. Tumor size 2 ~ 7 cm, an average of 4.3 cm, 9 cases of cystic degeneration. In addition to two cases of consultation, the total resection in 12 cases, subtotal resection in 3 cases. Follow-up of 10 cases, 8 cases of survival, survival time of 10 months to 13 years and 7 months, the average survival of 6 years, 10 patients survived in 2 cases. Histological features: mononuclear or multinucleated giant tumor cells, spindle cells and foam-like tumor cells mixed, the tumor is rich in reticular fibers and lymphocytes infiltration, the lack of necrosis, mitotic elephant or less. Glial fibrillary acidic protein, vimentin and S-100 protein immunohistochemical staining showed diffuse positive expression, the positive rate of CD34 was 77%. One case of PXA associated with anaplastic characteristics had more mitoses (≥5 / 10 HPF). 2 cases of brain parenchymal and perivascular space infiltration. One case of imaging examination revealed tumor recurrence and meningeal dissemination. Conclusion PXA is a WHO grade II tumor. Tumor resection and histology are typical of PXA with good prognosis. Minority of PXA may recur and ask questions. Giant tumor cells, weird, easily misdiagnosed as WHO Ⅳ grade giant cell glioblastoma, the main points of identification between the two parts of the visible part of the PXA foam cells, mitotic no or less, the lack of necrosis. The positive expression of tumor cell CD34 contributes to the diagnosis of PXA.