临床神经病学：22-1 颅内肿瘤

1、 Intracranial Tumors Primary Arising from intracranial tissues Metastatic Transfered from other system or organs Adult： Account for 1.8% of all cancers Children: 20% 胶质瘤 脑膜瘤脑膜瘤 垂体瘤 Epidemiology studies have not indicated any particular factors( viral, chemical or traumatic) that cause brain tumors i

2、n humans although a range of cerebral tumor can be induced in animals experimentlly. Aetiology Classification of brain tumors (WHO, 2007) 1.1.神经上皮组织肿瘤神经上皮组织肿瘤 Tumors of neuroepithelial tissue Tumors of neuroepithelial tissue 1 星形细胞瘤 Astrocytoma 2 少枝胶质细胞肿瘤OligodendroglialOligodendroglial tumors tumor

3、s 3 室管膜细胞肿瘤 Ependymal tumors 2 2 颅神经和脊旁神经肿瘤 Tumors of cranial and paraspinal Nerves 雪旺氏细胞瘤(神经鞘瘤) Schwannoma (Neurilemmoma) 3 脑膜组织肿瘤 Tumors of the Meninges 脑膜瘤 Meningioma 4 淋巴瘤和造血细胞肿瘤 5 生殖细胞肿瘤 Germ Cell Tumors 6 鞍区肿瘤 Tumors of the Sellar Region 垂体腺瘤 Pituitary adenoma 7 转移性肿瘤 Metastatic Tumors Epidemi

4、ology of brain tumor Epidemiology of brain tumor Primary intracranial tumorPrimary intracranial tumor Incidence 7.8-12.5/100 thousands Incidence 7.8-12.5/100 thousands Average 10/100 thousands Average 10/100 thousands Metastatic tumorMetastatic tumor Incidence 2.1-11.1/100 thousands Incidence 2.1-11

5、.1/100 thousands Relative Incidence 0 5 10 15 20 25 30 35 40 45 glioma meningioma pituitary adenoma schwannoma congenital tumor vascular tumor metastatic tumor others The distribution of brain tumor on ages 0 10 20 30 10 20 30 40 50 60 70 age The distribution of glioma on age (%) Presentation of bra

6、in tumor The most common presentation of brain tumor is progressive neurologic deficit(68%), usually motor weakness(45%). Symptoms and signs of increased intracranial pressure ： headache vomiting optic disc edema Focal neurologic deficits associated with brain tumors .1 those due to increased ICP A.

7、 from mass effect of tumor and /or edema B. from blockage of CSF drainage (hydrocephalus) Signs and symptoms include 2 progessive focal deficits: include weakness, dysphasia(which 37%-58% of patients with left-sided brain tumor) A due to destruction of brain parenchyma by tumor invasion B due to com

8、pression of brain parenchyma by mass and/or peritumoral edema C due to compression of cranial nerve 3. Headache 4 seizures: not infrequently the first symptom of a brain tumor Tumor should be aggressively sought in an idiopathic first time seizure in patient 20 years ( if negative, the patient shoul

9、d be followed with repeat studies at later dates） 5 Mental status changes depression lethargy apathy 6. Symptoms suggestive of a TIAor stroke may be due to : A . occlusion of a vessel by tumor cells B . hemorrhage into the tumor : any tumor may hemorrhage , C . focal seizure 7 in the special case of

10、 pituitary tumor A symptoms due to endocrine disturbances B pituitary apoplexy C CSF leak Focal neurologic deficits associated with brain tumor In addition to nonfocal signs and symptoms ( seizures, increased ICP) 1.Frontal lobe: abulia, dementia, personality changes. Often nonlateralizing, but apra

11、xia, hemiparesis or dysphasia( with dominant hemisphere involement ) may occur 2.Temporal lobe: auditory or olfactory hallucinations, memory impairment. Contralateral superior quadrantanopsia may be detected on visual field testing. 3. Parietal lobe: contralateral motor or sensory impairment, homony

12、mous hemianopsia, Agnosias (with dominant hemisphere involvement) and apraxias may occur 4. Occipital lobe: contralateral visual field deficit, alexia(especially with corpus callosum involvement with infiltrating tumors 5. Posterior fossa : cranial nerve deficits, ataxia(truncal or appendicular ) Di

13、agnosis of a brain tumor The diagnosis of brain tumors include three steps A Whether B What C Where 1 Clinical diagnosis history and signs 2 Image investigations CT MRI DSA PET Differentiate Diagnosis brain abscess parasitic infection brain hemorrhage brain infarction benign increased ICP Treatment

14、of brain tumors 1 Relieve increased ICP 2 Operation 3 Radiation therapy 4 Chemotherapy 5 GAMMA-knife Glioma Glioma comprise the majority of brain tumors and arise from the neuroglial cells derived from neuroectodermal origin There are four types of glial cells : Astrocytes Oligodendroglia Ependymal

15、cells Neuroglial precursor Astrocytoma (WHO grade I or II) The most common neuroepithelial tumor invade diffusely and have no distinct tumor margin. Location ： may arise in any part of the brain Adult ：cerebrum Children ：cerebellum Anaplastic astrocytoma, Glioblastoma (Malignant, WHO III orIV) ) gro

16、w more aggressively and can invade more cerebral lobes, even invade over midline. Astrocytoma Glioblastoma Clinical presentation Raised ICP Focal neurological signs Epilepsy Diagnosis Clinical presentation CT MRI Treatment : Operation : primary Radiation therapy: prolong the survival time Chemothera

17、py Prognosis：depend on the location of the tumor Median survival Astrocytoma: aboute over 5 years Anaplastic : 2-3 years glioblastoma: 1 years Medulloblastoma One of the most malignant brain tumor ( may seed in the subarachnoid cavity) location ：cerebelar vermis age：before 10 years old Clinical pres

18、entation： Increased ICP Ataxia Teatment：surgery radiation therapy chemotherapy Prognosis：5-year survival is above 30%以上，the best 80%. Meningioma Arise from arachnoid (not dura), slow growing, extra-axial, usually benign, circumscribed(non-infiltrating). Account for 14.319% Peak incidence 45 years ag

19、e Female:male ratio 1.8 : 1 Meningioma 1。May occur anywhere that arachnoi cells are found, most commonly located along falx, convexity, or sphenoid bone 2。Usually cured (if completely removed, which is not always possible ）. 3。Malignant incidence 1.7% of meningiomas 4。Multiple 8% of cases (more comm

20、on in Neurofibromatosis) Treatment Surgery Radiation therapy Ineffective as primary modality of treatment Efficacy in preventing recurrence is controversial Outcome 5 year survival for patients with meningioma 91.3% Pituitary adenoma Pituitary adenoma arise from the anterior gland (adenohypophysis)

21、May be classified by endocrine function routine histological staining method electron microscopic apperance Microadenoma: a pituitary adenoma 1cm diameter Pituitary adenoma Clinical presentation of pituitary adenoma Functional Non-functional Functional 1. Prolactin(PRL) : Prolactinomas are the most

22、common secretory adenoma. Amenorrhea-falactorrhea syndrome(Forbes- Albright) in females Impotence in males 2. Adrenocorticotropic hormone （ACTH) Cushings disease : endogenous hypercortisolism 3. Growth hormone(GH) Acromegaly in adults Gigantism in children 4. Rare pituitary adenomas secrete thyrotro

23、pin (TSH) : produce thyrotoxicosis gonadotropins (LH or FSH): no clinical syndrom Non-functional pituitary adenoma Mass effects of pituitary adenomas 1.Optic chiasm: classically resulting in bitemporal hemianopsia 2.Pituitary gland:resulting in varying degrees of hypopituitarism A. Hypothyroidism: c

24、old intolerance, myxedema, coarse hair B. Hypoadrenalism: orthostatic hypotension, easy fatigability C. Hypogonadisn: amenorrhea, loss of libido, infertility D. Diabetes insipidus E.Hyperprolatctinemia: PRL is under inhibitory control from the hypothalamus Diagnosis of pituitary adenomas History and