第246课（中枢神经）病例探析（069）—间变性少突胶质瘤

第246课（中枢神经）病例探析（069）—间变性少突胶质瘤男，66岁，突发头痛伴2月余
最后诊断：间变性少突胶质瘤
病理与临床特点
间变性少突胶质瘤( anaplastic oligoden droglioma,AO)即恶性少突胶质瘤,2016年WHO 新分类中属于弥漫性星形细胞与少突胶质细胞肿瘤,包括IDH 突变型与NOS型,分级为Ⅲ级,占胶质细胞肿瘤的25%~35%及所有原发脑肿瘤的1%-2%。

较少突胶质瘤易出现坏死。

镜下可见局限性或弥漫性恶性肿瘤征象,如肿瘤细胞密集、核异型性明显、核浆比増大,常见囊变、坏死及肿瘤细胞假栅栏状排列,肿瘤微血管增生明显。

免疫组织化学染色Ki-67明显增高,超过7%-10%。

发病年龄较少突胶质瘤大7-8岁,好发年龄为45~50岁。

临床表现与少突胶质瘤近似,最常见的症状为抽搐与头痛。

治疗首选手术切除,化疗可能有效,放疗用于治疗肿瘤残留与复发。

AO 预后欠佳,平均生存期为4年。

Anaplastic oligoden droglioma (anaplastic oligoden droglioma, AO) is less malignant glioma, in 2016 the WHO classification belongs to diffuse astrocytes and oligodendrocytes in tumor, including IDH mutation and NOS, class for Ⅲ, account for 25% ~ 35% of glial cells in the tumors and the 1% 2% of all primary brain tumors. Less prominent gliomas are prone to necrosis. Microscopically, there are signs of localized or diffuse malignancy, such as dense tumor cells, prominent nuclear atypia, enlarged nuclear plasma ratio, common cystic degeneration, necrosis, pseudopalisades of tumor cells, and prominent tumor
microvascular hyperplasia. Immunohistochemical staining significantly increased Ki-67 by more than 7%-10%. The age of onset is less than 7-8 years old, and the age of onset is 45-50 years old. The clinical manifestations are similar to oligodendroglioma. The most common symptoms are convulsion and headache. Surgical resection is preferred for treatment, chemotherapy may be effective, and radiotherapy is used to treat tumor residue and recurrence. AO has a poor prognosis with an average survival of 4 years.
CT与 MRI 特点
①部位及形态:AO 与少突胶质瘤好发部位类似,也以幕上、特别是额叶最常见,颞叶次之。

肿瘤可经胼胝体越过中线侵犯对侧大脑半球。

常为混杂密度或信号的大肿块,瘤周水肿、出血、囊变均较常见。

占位征象明显,如局部脑回增粗、脑沟及脑裂变窄、脑室受压,以及中线结构向对侧移位;
(1) location and morphology :AO and oligodendroglioma prone sites similar, also with the supratentorial, especially the frontal lobe is the most common, followed by the temporal lobe. Tumors may cross the midline through the corpus callosum and invade the contralateral hemispheres. Often be mixed density or signal big bump, hematoma week is oedema, haemorrhage, cystic change are more common. There were obvious signs of occupation, such as local gyri thickening, narrow fissions in sulcus and brain, ventricular compression, and contralateral migration of midline structure.
②CT 呈等、低及高密度混杂,其中低密度可为囊变/坏死区,高密度为出血或钙化,瘤周水肿为指状低密度,可侵犯内囊前后肢及外囊;
(2) CT showed a mixture of equal, low and high density, among which the low density could be the cystic/necrotic area, the high density could be the hemorrhage or calcification, and
the peritumor edema could be the fingerlike low density, which could invade the anterior and posterior limbs of the inner capsule and the outer capsule.
③MRI 呈混杂信号肿物,T1WI以等及低信号为主,囊变区为更低信号,灶周水肿呈指状或斑片状低信号,出血为高信号,压脂后信号不下降。

T2WI及 FLAIR以高信号为主,出血及钙化低信号;
(3) MRI showed mixed signal mass,T1WI was dominated by equal and low signal, the cystic area was lower signal, perifocal edema showed fingerlike or patchy low signal, bleeding was high signal, and the signal did not decrease after lipid compression. T2WI and FLAIR have hyperintensity, bleeding and low calcification.
④CT与MRI 増强扫描呈不同程度强化,实性部分强化明显,呈块状、斑片状、环形,以环状为主。

但也可不岀现强化。

总的来说,AO 强化率为62%~100%,明显高于少突胶质瘤。

且强化程度更明显,肿瘤增强比大(如1.30);
(4) CT and MRI enhanced scans were enhanced to different degrees, and the solid part was enhanced obviously, which was lumpy, patchy, annular, mainly annular. But they don't intensify immediately. In general, the AO enhancement rate was 62%~100%, which was significantly higher than oligodendyma. The enhancement degree was more obvious, and the tumor enhancement ratio was larger (e.g. 1.30).
⑤功能成像:CBV可增高。

MRS显示Cho峰及Cho/Cr明显增高(后者>2.33)、NAA降低。

T2*WI 及SWI显示出血及钙化为低信号。

(5) Functional imaging :CBV can increase. MRS showed that Cho peak and Cho/Cr increased significantly (>2.33), and NAA decreased. T2*WI and SWI showed low signal of hemorrhage and calcification.
鉴别诊断
①少突胶质瘤,仅从影像学上难以与AO鉴别,前者水肿、囊变、坏死及出血少见,而钙化较多见,MRS显示Cho峰及Cho/Cr 较低,增强T1WI对鉴别有一定价值,AO 强化更明显,典型者为厚壁、不规则花环状,但两者均可不出现强化,因此最终往往需病理学检查鉴别;
(1) gliomas, less with AO on imaging alone is difficult to identify, edema of the former, capsule, necrosis and hemorrhage rare, and calcification is more see, MRS showed lower peak and Cho Cho/Cr, enhanced T1WI of identification has a certain value, the AO strengthened more apparent, typical for thick wall, irregular flower ring, but may not appear both reinforcement, so often end to pathology inspection identification;
②少突-星形细胞混合胶质瘤及间变性星形细胞瘤或胶质母细胞瘤,仅从部位、影像学征象上难以鉴别,常需病理学检查确定诊断。

(2) oligodendrogate-astrocytoma mixed glioma and anaplastic astrocytoma or glioblastoma are difficult to distinguish only from the site and imaging signs, and pathological examination is often needed to confirm the diagnosis.
简要讨论
间变性少突胶质瘤较少见,其特点为发病年龄较大,瘤周水肿、瘤内坏死/囊变、出血较多见,而钙化较少,增强扫描肿瘤强化比増大,以不规则环状较有特征,MRS显示Cho峰明显增高。

但因其发病部位及形态特点类似于更常见的少突胶质瘤,因此术前诊断有一定难度,需综合多种征象甚至Ki-67才能定性诊断。

Anaplastic oligodendrogenic glioma is relatively rare, characterized by older onset age, more peri-tumor edema, intra-tumor necrosis/cystic degeneration, and bleeding, and less calcification, which is characterized by irregular ring and significantly increased Cho peak in MRS. However, due to its location and morphological characteristics similar to the more
common oligodendroglioma, preoperative diagnosis is difficult, and qualitative diagnosis can only be made by integrating multiple signs and even ki-67.
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