Meningioma
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Meningioma

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Meningioma Meningioma Presentation Transcript

  • Meningioma 신경외과 김선환 2001 년 7 월 26 일
  • General Consideration
    • 전체 원발성 뇌종양의 약 20%
    • 수막의 arachnoid cell 로부터 기원하는 양성의 extra-axial solid tumor
    • 중년의 여성에서 호발
    • 증상과 치료의 성적은 발생위치와 크기에 따라 다르다
    • 수술적 적출로 완치가 가능
  • Incidence
    • Population
      • Cranial: 0.3-8.4 per 100,000
      • Spinal: 0.08-0.3 per 100,000
    • 전체 원발성 뇌종양의 20%(13-40%)
    • 30% of incidental tumor at autopsy
    • Male-to-female ratio; Cranial 1:2, Spinal 1:5
    • more common at African(30%) vs others(15-20%)
    • Peak at middle age(esp. 5 th decade), rare at childhood(no female dominant)
    • Multiple : 1-9%
    • Malignancy: 2-12%
  • Origin
    • Arachnoid cell 에서 기원 : tela choroidea, choroids plexus, arachnoid cap cell
    • skull, spinal canal, choroid plexus and the arachnoid around cranial nerves
  • Etiology I
    • Trauma
      • brain abscess 와 meninges 의 만성 염증과정 ( 수십년전 받은 두부 외상에 의한 ) 이 수막종의 생성과 직접적인 관련이 있다는 보고가 있음 .
    • Virus
      • papova virus(simian virus40, BK, simian 40-like virus), adenovisus
      • viral protein, DNA, RNA: tumor induction 이나 transformation 의 유지에 역할을 하는것으로 생각
    • Radiation:
      • 1.6%, higher dose, younger patients
      • 직 , 간접적으로 DNA 손상을 가하여 neoplastic transformation 을 야기함
  • Etiology II
    • Genetics & molecular biology:
      • neurofibromatosis type II(chromosome 22 partial loss-tumor suppressor gene)
      • Type I (von Recklinghausen's disease)-less frequent
      • Oncogene 인 c- myc , c- fos , c- erb , h- ras , c- sis 의 mRNA 가 수막종에서 증가되며 특히 c-myc gene 은 atypical 이나 malignant meningioma 의 proliferation 과 연관되어 있는 것으로 추정
    • Hormone and growth factor receptors:
      • steroid hormone 과 polypeptide 가 수용체와 결합하면 cellular proliferation 을 유발
      • 수막종에서 progesterone receptor 발견 가능성 높음
  • Pathology: Macroscopic features
    • Dura 에 부착되어 있으며 보통 구형이며 capsule 로 덮여 있다 .
    • Dura 아래로 자라 brain parenchyme 을 압박하나 악성을 제외하고는 infiltration 은 없다 .
    • Dura 로부터 쉽게 박리되나 venous sinus 침범을 종종 볼 수 있다 .
    • 종양은 보통 고무와 비슷하게 단단하나 때로는 연하고 드물게 cystic degeneration 이 나타남
    • hypervascularity, peritumoral edema 가 종종 관찰되며 hyperostosis 가 5% 정도에서 관찰
    • typical meningioma 는 hypervascular nature 로 dural artery 로부터 혈류공급
  • Pathology: Macroscopic features
  • Pathology: Macroscopic features Hyperostosis of skull Bony erosion
  • Pathology: WHO classification(1993)
    • Neuroepithelial Tumors of the CNS
      • Astrocytic tumors
      • Oligodendroglial tumors
      • Ependymal cell tumors
      • Mixed gliomas
      • Neuroepithelial tumors of uncertain origin
      • Tumors of the choroid plexus
      • Neuronal and mixed neuronal-glial tumors
      • Pineal Parenchyma Tumors
      • Tumors with neuroblastic
    • Other CNS Neoplasms
      • Tumors of the Sellar Region
      • Hematopoietic tumors
      • Germ Cell Tumors
      • Tumors of the Meninges
        • Meningioma
      • Non-menigothelial tumors of the meninges
      • Benign Mesenchymal
      • Malignant Mesenchymal
      • Primary Melanocytic Lesions
      • Hemopoietic Neoplasms
      • Tumors of Uncertain Histogenesis
      • Tumors of Cranial and Spinal Nerves
      • Local Extensions from Regional Tumors
      • Metastatic tumours
      • Unclassified Tumors
      • Cysts and Tumor-like Lesions
    • Meningioma
        • variants:
        • meningothelial,
        • fibrous (fibroblastic )
        • transitional (mixed)
        • psammomatous,
        • angiomatous, microcystic,
        • secretory, clear cell,
        • chordoid, metaplastic
        • lymphoplasmacyte-rich,
    • Atypical meningioma
    • Anaplastic (malignant) meningioma
  • Pathology: Russell classification Papillary Malignant Hemangioblastic Transitional Hemangiopericytic Angioblastic Psammomatous, Microcystic Myxomatous, Xanthomatous Lipomatous, Granular Secretory, Chondroblastic Osteoblastic, Melanotic Giant cell, Lymphoid, Choroids Meningothelial Transitional Fibrous Classic Variants Subtype Group
  • Pathology: Microscopic features
    • Meningothelial meningioma: 경계가 불분명한 polygonal cell 이 vascular trabeculae 에 의해 간격지어져 syncytial appearance 를 보인다 .
  • Pathology: Microscopic features
    • Fibrous meningioma: spindle shaped cell 과 prominent interstitial collagen 으로 이루어짐
  • Pathology: Microscopic features
    • Transitional meningioma: meningothelial 과 fibrous meningioma 의 혼합형태로 concentric whorl formation 이 보인다 .
  • Pathology: Microscopic features
    • Psammomatous meningioma: calcified psammoma body 가 많다
  • Image: Plain X-ray
    • Tumor calcification(10%)
    • Hyperostosis
  • Image: CT
    • Non-contrast CT 에서 well circumscribed rounded mass 로 dural surface 에 broad implantation 을 보인다 .
    • 대부분 brain 에 비하여 isodense 혹은 slight hyperdense 한 음영을 보이며 조영제에 homogenous enhance 되어 hyperdense 소견을 보인다 .
    • calcification, hyperostosis, bony erosion, cystic nature
  • Image: CT Pre-enhance CT Post-enhance CT
  • Image: MRI
    • Extra axial mass 로 normal brain parenchymal compression
    • T1 weighted image: 60%-isointense to gray matter, 30%-slight hypointense signal
    • T2 weighted & proton density image: 50%-isointense to gray matter, 40%-slight hyperintense signal
    • 조영제에 Homogenous strong enhance
    • dural tail sign: dural thickening with enhancement
    • peritumoral edema: 46-92%,
      • T2 image 에서 high signal intensity
  • Image: MRI T1-Pre enhance T1-Post enhance T2WI
  • Image: Cerebral Angiography
    • CT 와 MRI 에 추가하여 진단의 재평가와 수술계획수립에 중요
    • 전형적으로 hypervascular tumor 로 대게 dural vessel 로부터 blood supply 받음
    • sunburst appearance : enlarged dural feeder 와 intense tumor blush 로 인함
    • tumor staining
  • Image: Cerebral Angiography
  • Location
    • 90% 가 천막상부에 발생한다 .
    • parasagittal(25-30%) > convexity(20%)
    • > sphenoid ridge(15-20%)
    • > others(olfactory groove, tuberculum sellae, falx, CP angle, foramen magnum, tentorial, petrosal, middle cranial fossa, ventricles)
  • Location
  • Clinical Manifestation
    • compression of brain parenchyma and cranial nerve: location & growth rate 에 따라 다양
    • seizure: about 50%, focal seizures at motor cortex; mass irritation 이 관계
    • vascular event:
      • dural venous sinus occlusion
      • bleeding(about 5%)- headache, IICP Sx, mental change
  • Clinical Manifestation by Specific location Change in mentation, apathy or disinhibited behavior, urinary incontinence Subfrontal Visual Sx, 3 rd N palsy, Hypopituitarysm, Psychic sx, headache Tuberculum sellae Headache, seizure, focal weakness Convexity Ant 1/3: personality change Mid 1/3: contralateral leg Monoparesis Post 1/3: IICP Sx, visual field defect Parasagittal Symptoms Location
  • Clinical Manifestation by Specific location Symptoms Location Decreased hearing with possible facial weakness and facial numbness Cerebellopontine angle Contralateral hemianopsia Occipital lobe Multiple cranial N deficits (II, III, IV, V and VI), leading to decreased vision and diplopia with associated to facial numbness Cavernous sinus Anosmia with ipsilateral optic atrophy and contralateral papilledema; Kennedy-Foster syndrome. Olfactory groove
  • Clinical Manifestation by Specific location Symptoms Location Paraparesis; stereoanesthesia, gait disturbance Foramen magnum supratentorial and infratentorial; seizure, cerebellar sx, hydrocephalus, visual sx. Tentorium Medial 1/3(clinoidal): multiple cranial nerve palsies Middle 1/3(alar): headache, seizure Lateral 1/3(pterional): seizure, palpable mass Sphenoid wing Exophthalmos, Monocular loss of vision; ipsilateral dilated pupil orbital
  • Management: Surgery
    • 종양의 완전적출로 완치가능 - 치료에 가장 중요함 .
    • 원칙
      • 충분한 노출 : careful positioning of patient, well planned incision
      • 종양으로 가는 혈류의 가능한 조기 차단
      • cavitron, bipolar coagulation 등을 이용한 internal decompression
      • tumor capsule 의 careful dissection 과 주위 뇌조직의 minimizing retraction
      • 가능한 침범된 dura 와 bone 의 제거
      • dural defect 의 pericranial tissue 나 인조 경막을 이용한 reconstruction
  • Management: Radiation Therapy
    • External beam radiation therapy
      • 완전 적출이 불가능한경우
        • old age
        • 전신상태가 수술에 적응하기 어려운 경우
        • deep positioned tumor: cavernous sinus, petrosal, tentorial, clival etc
      • malignant meningioma, hemangiopericytoma
      • recurrence of tumor
    • Radiosurgery -linear accelerator, Gamma knife, proton beam
    • Intersititial brachytherapy -recurrent & malignant, Iodine-125
  • Management: Chemotherapy
    • Cytotoxic chemotherapy: little experience
    • Hormone receptor antagonists: antiprogesterone agents(MPA, mifepristone, RU38486)
  • Recurrence & Prognosis
    • Resectability- Simpson grade by extent of removal
      • Complete remove with dura & bone- 9%
      • Complete remove with dura coagulation-16%
      • Only mass remove-29%
      • Partial remove- 39%
      • Only biopsy- 100%
    • Type- atypical, malignant, papillary, hemangiopericytoma; recur 증가
    • mortality 가 증가하는 경우 :
      • Old age, deep location, reoperation by tumor recurrence
  • Malignant meningioma
    • 전체 수막종의 2-12%, 남자 > 여자 , 50 대에 호발
    • Parasagittal & convexity(70%), rare at post.fossa
    • 뇌실질의 침윤 , mitoses, necrosis, nuclear & cytoplasmic pleomorphism, papillary type
    • liver, lung, lymph node, bone, pleura 등으로 전이가 가능하며 뇌척수액을 통한 전이는 상대적으로 적다 .
    • CT: indistinct tumor margin, central low density, peritumoral edema
    • MRI: T2WI 에서 signal 의 증가 , poorly defined margin, 광범위한 edema, bone destruction 과 bony foramen 을 통한 extension 이 보일 때 의심
    • 완전적출후에도 재발가능성이 높고 chemotherapy 는 효과가 없다 .
    • Medial survival time: 2 년 정도
  • Multiple meningioma
    • 전체 수막종의 1-9%, 여성에서 더 흔하며 (93%), 평균 연령은 50 세 전후
    • supratentorial convexity, parasagittal, falx 등에 주로 발생
    • neurofibromatosis 와 잘 동반
    • 한쪽 대뇌 반구만 침범되는경우가 50%, 종양이 2 개인경우가 71.4%
  • Meningioma in children
    • 전체 뇌종양의 1-4%
    • no female predominance
    • supratentorial(67%), infratentorial(14.4%), intraventricular(9.4%), orbital(5.4%)
    • 성인에 비하여 malignant 가 흔함
    • 성인에 비하여 예후 불량 : 종양의 크기가 크고 , 악성변화가 많고 , unusual location 발생