神經(jīng)節(jié)膠質(zhì)瘤( ganglioglioma,GG)是一種來(lái)自于發(fā)育不良性神經(jīng)節(jié)細(xì)胞與膠質(zhì)細(xì)胞的良性腫瘤,屬于神經(jīng)元與神經(jīng)膠質(zhì)混合腫瘤。僅占原發(fā)腦腫瘤的1%-1.5%及兒童腦腫瘤的5%-10%。一般為WHOl級(jí),少數(shù)為WHOⅡ級(jí)、甚至間變(Ⅲ級(jí))。病理學(xué)上腫瘤呈灰棕色,局部皮質(zhì)增厚。鏡下可見(jiàn)神經(jīng)元與神經(jīng)膠質(zhì)兩種成分,神經(jīng)元分化不良,膠質(zhì)細(xì)胞增生及異型,腫瘤性膠質(zhì)細(xì)胞以毛細(xì)胞樣或纖維性最常見(jiàn), 也可為少突膠質(zhì)細(xì)胞。血管周?chē)?jiàn)淋巴細(xì)胞浸潤(rùn)。腫瘤間變少見(jiàn),MIB-1為1%-3%。免疫組織化學(xué)染色顯示神經(jīng)與膠質(zhì)來(lái)源兩種特征,CFAP、突觸素及神經(jīng)微絲蛋白(+),CD34可為(+)。好發(fā)于青年及兒 童,80%于30歲前發(fā)病,無(wú)明顯性別差異。最常見(jiàn)臨床表現(xiàn)為顳葉難治性癲癇。手術(shù)切除為首選治療方法。預(yù)后良好。
Ganglioglioma (GG) is a kind of benign tumor derived from underdeveloped ganglion cells and glial cells. Only 1% to 1.5% of primary brain tumors and 5% to 10% of childhood brain tumors. Levels for WHOl commonly, for minority WHO Ⅱ, even change (Ⅲ level). Pathologically, the tumor is grayish brown with local cortical thickening. Microscopically, there are two components of neurons and glia. Neurons are poorly differentiated, glial cells proliferate and are abnormal. Neoplastic glial cells are most common in hair cell-like or fibrous form, and can also be oligodendrocytes. Perivascular infiltration of lymphocytes is seen. Intertumor degeneration was rare, and MIB-1 was 1%-3%. Immunohistochemical staining showed two characteristics of nerve and glial origin,CFAP, synapse and neurofilaggrin (+),CD34 could be (+). It is most common in young people and children. 80% of cases occur before the age of 30. The most common clinical manifestation is temporal lobe intractable epilepsy. Surgical resection is the preferred treatment. The prognosis is good.
臨床癥狀決定于腫瘤所在部位,主要臨床癥狀為癲癇,后期可出現(xiàn)顱內(nèi)壓增高癥狀。腫瘤部位不同,癥狀不同,位于顳葉的腫瘤常有癲癇發(fā)作,屬顳葉相關(guān)性癲癇,若腫瘤位于枕葉,可出現(xiàn)視力異常。由于腫瘤生長(zhǎng)緩慢,臨床病史一般較長(zhǎng),病史從1個(gè)月到50年,平均25年。節(jié)細(xì)胞膠質(zhì)瘤常因藥物不能控制的癲癇而做大腦皮層手術(shù),占癲癇手術(shù)的15%-25%,是與慢性顳葉癲癇相關(guān)的最常見(jiàn)的腫瘤。
Clinical symptoms depend on the location of the tumor. The main clinical symptoms are epilepsy. Tumor site is different, symptom is different, the tumor that is located in temporal lobe often has epileptic seizure, belong to temporal lobe relevance epileptic, if tumor is located in occipital lobe, can appear eyesight is abnormal. Due to slow tumor growth, clinical history is generally long, ranging from 1 month to 50 years, with an average of 25 years. Ganglion glioma is the most common tumor associated with chronic temporal lobe epilepsy.
腫瘤通常境界清楚,常見(jiàn)單個(gè)囊并伴有壁結(jié)節(jié),鈣化常見(jiàn)。顯微鏡下節(jié)細(xì)胞膠質(zhì)瘤由不規(guī)則簇狀、發(fā)育不良的大多極神經(jīng)元和膠質(zhì)細(xì)胞構(gòu)成,膠質(zhì)細(xì)胞主要為星形細(xì)胞,偶爾可見(jiàn)少突膠質(zhì)細(xì)胞,腫瘤性膠質(zhì)成分和網(wǎng)織纖維網(wǎng)圍繞星形細(xì)胞排列。病變可擴(kuò)展到附近腦膜,膠質(zhì)成分可在腫瘤細(xì)胞和間質(zhì)之間浸潤(rùn)性生長(zhǎng)。少許核分裂象不影響節(jié)細(xì)胞膠質(zhì)瘤的診斷,膠質(zhì)成分惡變時(shí)可有壞死。血管周?chē)Ｒ?jiàn)淋巴細(xì)胞浸潤(rùn)。
Tumors are usually well defined, often with a single capsule and wall nodules, and calcification is common. Microscopically, a ganglion glioma is composed of irregular clusters of poorly developed large multipolar neurons and glial cells, mainly astrocytes, with occasional oligodendrocytes. Neoplastic glial components and reticular fibers line the astrocytes. Lesions may extend to the adjacent meninges, and glial components may infiltrate between tumor cells and stroma. A small amount of mitotic appearance does not affect the diagnosis of ganglion glioma. Perivascular lymphocyte infiltration is common.
CT典型的節(jié)細(xì)胞膠質(zhì)瘤表現(xiàn)為伴有壁結(jié)節(jié)的囊性病變,壁結(jié)節(jié)常見(jiàn)鈣化,囊性部分呈低密度。
The typical CT appearance of a ganglion glioma is a cystic lesion with a wall nodule.
MRI主要見(jiàn)于幕上大腦半球,以顳葉最常見(jiàn),其次是額葉和頂葉T1WI常表現(xiàn)為不均勻低信號(hào),T2WI一般表現(xiàn)為高信號(hào)。增強(qiáng)掃描半數(shù)以上囊壁有輕中度強(qiáng)化。囊壁岀現(xiàn)鈣化常提示該腫瘤。大部分節(jié)細(xì)胞膠質(zhì)瘤DWI信號(hào)高于正常腦組織,也可表現(xiàn)為等、稍低信號(hào),ADC值增高。
MRI is mainly found in the supratentorial cerebral hemisphere, with the temporal lobe being the most common, followed by the frontal lobe and parietal lobe. T1WI often presents uneven and low signal, while T2WI generally presents high signal. More than half of the capsule walls showed mild to moderate enhancement on enhanced scan. Report calcification of cystic wall often suggests this tumour. DWI signal of most ganglion glioma is higher than that of normal brain tissue, but it can also be expressed as isotropic or slightly lower signal, and ADC value is increased.
節(jié)細(xì)胞膠質(zhì)瘤的MR表現(xiàn)多樣,對(duì)于難治性癲癇的青少年患者,若發(fā)現(xiàn)腦內(nèi)皮層或皮層下白質(zhì)為主的病變,易囊變,瘤周水腫輕,強(qiáng)化程度多樣,要考慮神經(jīng)節(jié)細(xì)胞膠質(zhì)瘤的可能;而出現(xiàn)多形性膠質(zhì)母細(xì)胞瘤樣的強(qiáng)化方式時(shí)要警惕腫瘤間變的發(fā)生。典型的節(jié)細(xì)胞膠質(zhì)瘤表現(xiàn)為單個(gè)大囊加壁結(jié)節(jié)鈣化,腫瘤境界常清楚,一般容易診斷。囊變不明顯或呈多發(fā)小囊變者與節(jié)細(xì)胞瘤無(wú)法鑒別,實(shí)際上節(jié)細(xì)胞瘤和節(jié)細(xì)胞膠質(zhì)瘤的區(qū)別僅僅表現(xiàn)在組織學(xué)檢查上。需要與DNET、低級(jí)別星形細(xì)胞瘤、膠質(zhì)母細(xì)胞瘤等鑒別。
MR manifestations of ganglion glioma are diverse. For adolescent patients with refractory epilepsy, the possibility of ganglion glioma should be considered if lesions dominated by intracortex or subcortical white matter are found, which are prone to cystic degeneration, with mild peritubercular edema and diverse intensification degree. However, the occurrence of glioblastoma multiforme like enhancement should be wary of the occurrence of changes between tumors. A typical ganglion glioma is characterized by a single large cystic wall nodule calcification. In fact, the difference between a ganglion cell tumor and a ganglion glioma is only demonstrated by histological examination. It needs to be differentiated from DNET, low grade astrocytoma and glioblastoma.
DNET可見(jiàn)“三角征”、T2-FLAIR高信號(hào)環(huán)征、顱骨壓迫等征象,提示良性緩慢生長(zhǎng)的過(guò)程,但缺乏特異性。DNET囊內(nèi)分隔較多見(jiàn),有助于兩者的鑒別。
DNET showed signs of trigonometry, t2-flair ring, and skull compression, suggesting benign slow growth but lacking specificity. More compartments in DNET capsule are seen, which is helpful for the identification of DNET and DNET.
低級(jí)別星形細(xì)胞瘤發(fā)生于幕下時(shí),要和毛細(xì)胞型星形細(xì)胞瘤鑒別,MRS有助于鑒別診斷,星形細(xì)胞瘤的Cho峰升高較明顯,但神經(jīng)節(jié)細(xì)胞膠質(zhì)瘤的Cho峰僅輕度升高,NAA峰略降低。
When a low-grade astrocytoma occurs in the subtentorium, it should be differentiated from hair cell astrocytoma. MRS is helpful for differential diagnosis. Cho peak of astrocytoma is significantly increased, but Cho peak of ganglion glioma is only slightly increased, and NAA peak is slightly decreased.
膠質(zhì)母細(xì)胞瘤好發(fā)于中老年人,深部白質(zhì)區(qū),可沿胼胝體向?qū)?cè)半球延伸,呈蝶翼狀改變易壞死,周?chē)[明顯,增強(qiáng)檢查呈花環(huán)狀強(qiáng)化.
Glioblastoma usually occurs in middle-aged and elderly people. Deep white matter areas can extend along the corpus callosum to the contralateral hemisphere, presenting a sphenoid pterygoid change and easy necrosis, with obvious surrounding edema, and enhancement examination showed flower ring enhancement.
