CNS Tumors - clinical and radiological aspects.

Q4 Medicine Ceskoslovenska patologie Pub Date : 2022-01-01
Renata Emmerová, Jana Engelová, Stěpan Vinakurau, Barbora Ondrová
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Abstract

Tumors of the central nervous system (CNS) include primary tumors - itraaxial, growing from brain and spinal cord cells (neuroepithelial tumors) or extraaxial, growing from surrounding structures (brain and spinal cord, nerve sheaths, vascular structures, lymphatic tissue, germ cells, malformations, pituitary glands). Much more often they are located in the intracranial space a solitary or multiple metastatic spread of malignancy originating from another organ (eg lung, breast, malignant melanoma, Grawitzs tumor). The occurrence of metastases of solid tumors is then in the intraaxial or extraaxial region, leptomeningeal or dural. Even morphologically benign tumors with their occurrence in a closed CNS compartment can have malignant behaviour and cause severe slowly developing to acute neurological symptoms, including intracranial hypertension. Primary tumors of the central nervous system present 1-2% of all cancers, with a higher incidence in adults after the age of 60, with a slight predominance in men, with higher mortality in men than in women. About 5% of CNS tumors are hereditary (e.g., Li-Fraumeni syndrome, neurofibromatosis type I, II). The causes of most brain and spinal cord tumors are unclear, the effect of radiation has been definitely demonstrated, there is an increased risk in transplant patients and AIDS (Acquired Immune Deficiency Syndrome) patients, and the potentiating effects of some chemicals and viruses on the development of CNS neoplasms are uncertain. The effectiveness of treatment of brain and spinal cord tumors is influenced by the existence of the so-called hematoencephalic barrier, which protects the brain from the penetration of toxic substances, but at the same time prevents the penetration of most cytostatics to the tumor target. Another obstacle may be the localization of the tumor in areas difficult to access for histological verification (brain stem, optical chiasma) due to the high risk of complications even after stereotactic biopsy. In some cases, in an effort not to cause an irreversible neurological deficit by inconsiderate tissue collection, the sample of histological material can then become inconclusive to tumor cells, i.e., tumor cells are not captured. Last but not least, the radiosensitivity of some brain structures is also limiting, which makes it impossible to apply a higher dose of ionizing radiation to a tumor affecting sensitive tissues or located near of these sensitive tissues. The rapid development of immunohistochemical (IHC) and molecular genetic analysis methods has significantly refined diagnostics and thus theoretically facilitates the choice of the optimal treatment procedure for the individual patient. While advances in modern conformal photon and particle (currently the most frequently proton) radiotherapy, stereotactic radiosurgery has enabled accurately targeted irradiation of the CNS tumor site and at the same time spare the high-risk brain structures, thereby significantly reduce the risk of acute and late neurotoxicity, pharmacotherapy options are still limited. Just molecular-genetic knowledge already provides us with predictive and prognostic information. They should increasingly stratify patients for targeted therapy.

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中枢神经系统肿瘤-临床和放射学方面。
中枢神经系统(CNS)的肿瘤包括原发性肿瘤-轴内肿瘤,从脑和脊髓细胞生长(神经上皮肿瘤)或轴外肿瘤,从周围结构(脑和脊髓,神经鞘,血管结构,淋巴组织,生殖细胞,畸形,垂体)生长。更多情况下,它们位于颅内间隙,是源自其他器官的恶性肿瘤(如肺、乳腺、恶性黑色素瘤、格拉维茨瘤)的单发或多发转移性扩散。实体瘤的转移发生在轴内或轴外区域,脑膜或硬脑膜。即使形态上良性的肿瘤发生在封闭的中枢神经系统隔室中,也可能具有恶性行为,并引起严重的缓慢发展为急性神经系统症状,包括颅内高压。中枢神经系统的原发性肿瘤占所有癌症的1-2%,60岁以后的成年人发病率较高,男性略占优势,男性死亡率高于女性。大约5%的中枢神经系统肿瘤是遗传性的(如Li-Fraumeni综合征、I型、II型神经纤维瘤病)。大多数脑和脊髓肿瘤的病因尚不清楚,辐射的影响已得到明确证实,移植患者和艾滋病(获得性免疫缺陷综合征)患者的风险增加,一些化学物质和病毒对中枢神经系统肿瘤发展的增强作用尚不确定。脑和脊髓肿瘤治疗的有效性受到所谓血脑屏障存在的影响,血脑屏障保护大脑免受有毒物质的渗透,但同时阻止大多数细胞抑制剂渗透到肿瘤靶点。另一个障碍可能是肿瘤定位在难以进入组织学验证的区域(脑干,视交叉),因为即使在立体定向活检后并发症的风险很高。在某些情况下,为了避免因组织收集不当而导致不可逆转的神经功能缺陷,组织材料样本可能会对肿瘤细胞产生不确定性,即肿瘤细胞没有被捕获。最后但并非最不重要的是,某些大脑结构的辐射敏感性也有限,这使得不可能对影响敏感组织或位于这些敏感组织附近的肿瘤施加更高剂量的电离辐射。免疫组织化学(IHC)和分子遗传分析方法的快速发展大大改进了诊断方法,从而在理论上为个体患者选择最佳治疗方案提供了便利。虽然在现代适形光子和粒子(目前最常见的质子)放射治疗中,立体定向放射外科手术能够精确靶向照射中枢神经系统肿瘤部位,同时避免了高危脑结构,从而显着降低了急性和晚期神经毒性的风险,但药物治疗选择仍然有限。仅仅分子遗传学知识就已经为我们提供了预测和预后的信息。他们应该越来越多地对患者进行分层,以进行靶向治疗。
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来源期刊
Ceskoslovenska patologie
Ceskoslovenska patologie Medicine-Medicine (all)
CiteScore
0.40
自引率
0.00%
发文量
17
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