男，26岁，头痛伴头晕10余天
最后诊断：室管膜下巨细胞性星形细胞瘤
室管膜下巨细胞性星形细胞瘤(Subependymal giant cell astrocytoma，SGCA)也是一种WHO I级星形细胞 ,仅见于结节性硬化(心)病人,与室管膜下错构性结节密切相关。病理学上肿细胞变化多样,很难与 错构性结节区分,可见大锥体细胞,是梭形或上皮样,胞质丰富,胞核圆而大,染色质偏位,但很少间变 GFAP染色(+)SCCA仅见于1/5以下的TS病人,但一旦出现,约1/4出现神经系统症状。最常见的临 床表现为抽搐,其他包括恶心呕吐、意识障碍等本病进展慢,一般不侵犯脑实质,因此预后良好。阻 塞室间孔及并发出血者可致脑积水与猝死。
Subependymal giant cell astrocytoma (SGCA) is a WHO grade I astrocyte. It is only seen in patients with tuberous sclerosis (cardiac) and is closely related to the Subependymal hamartoma. Pathologically, the tumor cells are diverse and difficult to distinguish from the disorganized nodules. The large pyramidal cells are spindle or epithelioid, with abundant cytoplasm, round and large nuclei, and deviated chromatin, but rarely variable GFAP staining (+)SCCA is found in less than 1/5 of TS patients, but once present, about 1/4 of them present with nervous system symptoms. The most common bedside manifestations are convulsions, and other diseases such as nausea and vomiting and consciousness disorders progress slowly and generally do not invade the brain parenchyma, so the prognosis is good. Obstruction of interentricular pores and concurrent bleeding may lead to hydrocephalus and sudden death.
CT与MRI特点
①部位及形态,均发生于室间孔附近,偶有脑室外肿瘤的报道。肿物呈结节状、类圆形或分叶状。其 大小可从很小的点状到数厘米大小,一般为10-15mm,随访肿瘤常每年增大2.5mm-5.6mm。多数单发约1/5病例多发。肿瘤内少见出血及坏死,但钙化常见。15%合并双侧侧脑室扩大;
(1) location and morphology, all occurred near the interventricular foramen, and there were occasional reports of tumors outside the ventricle. The tumor was nodular, circular or lobulated. The size can range from a small dot to a few centimeters, usually 10-15mm, and the tumor usually increases by 2.5mm to 5.6mm per year during follow-up. Most single cases occurred in about 1/5 cases. Hemorrhage and necrosis are rare, but calcification is common. 15% with bilateral ventricular enlargement;
②CT平扫肿物为等 或稍低密度,常伴结节状高密度影,同时合并侧脑室周围钙化,尤其常见于丘脑纹状体沟。增强扫描呈不均匀及显著强化;
(2) the mass on plain CT scan was of equal or slightly lower density, often accompanied by nodular high-density shadow, and accompanied by periventricular calcification, especially in the striatum sulcus of thalamus. The enhancement scan showed non-uniform and significant enhancement.
③肿瘤呈T1等或低信号、T2等至高信号,钙化结节呈T1稍高及T2低信号。 FLAIR呈略高信号,并有助于检出错构性病变,呈室管膜下向外延伸的条纹状或楔形高信号。增强扫描肿瘤强化程度高于CT所见；
(3) the tumor showed T1 or low signal, T2 to high signal, calcified nodules showed T1 slightly high signal and T2 low signal. FLAIR has a slightly hyperintensity and is useful for detecting structural lesions with a striated or wedge-shaped hyperintensity extending outward from the subependymal region. The enhancement degree of tumor in enhanced scan was higher than that in CT.
④非手术治疗的病人应2年左右复查一次颅脑影像学;
(4) non-surgical treatment of patients should be about 2 years to review the brain imaging;
⑤血管造影可见肿瘤一定程度染色。
(5) angiography showed a certain degree of tumor staining.
鉴别诊断
①主要是TSC的室管膜下非肿瘤性结节,这些结节长期稳定,无需切除,一般<5mm,可位于侧脑室旁 任何部位,丘脑纹状体沟最多见:
(1) mainly the TSC suboependymal non-neoplastic nodules, these nodules long-term stability, no need to remove, generally <5mm, can be located in any part of the lateral ventricle, thalamic striatum groove is the most common:
②室管膜下瘤,见于中老年病人,多无明显强化;
Subependymoma, seen in middle-aged and elderly patients, mostly without obvious enhancement;
③脉络丛乳头状瘤,位于第三脑室者可延伸至室间孔,5岁以下儿童多见,强化更为显著,多为分叶状,未造成梗阻者也可出现脑积水;
(3) choroid plexus papilloma, located in the third ventricle can extend to the interventricular hole, more common in children under 5 years old, the enhancement is more significant, mostly lobular, without causing obstruction can also appear hydrocephalus;
④中枢神经细胞瘤,常见青年女性,起源于侧脑室体部,多发囊变者呈泡泡状,强化较轻:
(4) central nerve cell tumor, common young women, originated from the lateral ventricle body, multiple cystic changes were bubble-shaped, mild enhancement:
⑤脑室内胶质瘤,常为邻近脑实质或透明隔肿瘤侵犯所致,伴瘤周水肿:
(5) intraventricular glioma, usually caused by invasion of adjacent brain parenchyma or transparent septum tumor, accompanied by peritumor edema:
⑥室管膜瘤,易侵犯脑实质,囊变及水肿明显
6. Ependymoma, easy to invade the brain parenchyma, cystic degeneration and edema is obvious
⑦少突胶质瘤,常位于脑实质表浅部位,可见粗大条状或弧形钙化;
7, less process glioma, often located in the superficial surface of the brain parenchyma, visible thick strip or arc calcification;
⑧脑室内脑膜瘤,三角区多见,信号及密度近似脑实质,增强扫描显著及均匀强化;
(8) intraventricular meningioma, trigonometry more common, signal and density approximate brain parenchyma, enhanced scanning significantly and uniform enhancement;
⑨转移瘤,有原发肿瘤史,见于40岁以上病人,可多发及瘤周水肿明显;
(9) metastatic tumor, with a history of primary tumor, seen in patients over 40 years old, with multiple tumors and obvious peritumor edema;
⑩毛细胞性星形细胞瘤,见于儿童或青年,呈囊实性,实性区域及囊壁明显强化
Astrocytoma, seen in children or young adults, is cystic with evident enhancement of the solid area and the wall of the cyst
简要讨论
SGCA具有特征性的临床与影像表现,如抽搐、智力减退、面部错构瘤(三联征),侧脑室室管膜多发结节伴钙化,肿瘤大部分位于室间孔附近,并可合并肾囊肿及肾细胞癌等
This paper briefly discusses the characteristic clinical and imaging manifestations of SGCA, such as convulsion, mental retardation, facial hamartoma (triad), multiple lateral ventricular ependymal nodules with calcification, most tumors located near the interventricular foramen, and renal cysts and renal cell carcinoma
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