大脳神経膠腫症
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出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2012/05/06 17:24:06」(JST)
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Gliomatosis cerebri |
Classification and external resources |
Axial fluid-attenuated inversion recovery MRI image demonstrating tumor-related infiltration involving both temporal lobes (Short arrow), and the substantia nigra (Long arrow). |
ICD-10 |
ICD9 = Template:ICD9 191.0 |
ICD-O: |
M9381/3 |
MeSH |
D018302 |
Gliomatosis cerebri (infiltrative diffuse astrocytosis) is a rare primary brain tumor. It is commonly characterized by diffuse infiltration of the brain with neoplastic glial cells that affect various areas of the cerebral lobes.[1] Glimatosis Cerebi behaves like a malignant tumor that is very similar to Glioblastoma.
While gliomatosis cerebri can occur at any age, it is generally found in the third and fourth decades of life.
Contents
- 1 Presentation
- 2 Diagnosis
- 3 Prognosis
- 4 Notes
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Presentation
It may affect any part of the brain or even the spinal cord, optic nerve and compact white matter. Clinical manifestations are indefinite, and include headache, seizures, visual disturbances, corticospinal tract deficits, lethargy, and dementia. A case of gliomatosis cerebri presenting as rapidly progressive dementia and Parkinson's disease like symptoms was described in an 82 year old woman.[2]
Diagnosis
Before the advent of MRI, diagnosis was generally not established until autopsy. Even with MRI, however, diagnosis is difficult.[3] Typically, gliomatosis cerebri appears as a diffuse, poorly circumscribed, infiltrating non-enhancing lesion that is hyperintense on T2-weighted images and expands the cerebral white matter. It is difficult to distinguish from highly infiltrative anaplastic astrocytoma or GBM.[4]
Prognosis
The prognosis for gliomatosis cerebri is generally poor, with a median survival time of only 12 months.[5] Surgery is not practical considering the extent of the disease, standard chemotherapy (nitrosourea) has been unsuccessful, and while brain irradiation can stabilize or improve neurologic function in some patients, its impact on survival has yet to be proven.[citation needed]
Notes
- ^ Gliomatosis Cerebri, MedPix : 923 - Medical Image Database and Atlas
- ^ http://jmedicalcasereports.com
- ^ Bendszus M, Warmuth-Metz M, Klein R, et al. (2000). "MR spectroscopy in gliomatosis cerebri". AJNR Am J Neuroradiol 21 (2): 375–80. PMID 10696026. http://www.ajnr.org/cgi/pmidlookup?view=long&pmid=10696026.
- ^ "Radiology, University of Rochester Medical Center". http://www.urmc.rochester.edu/smd/Rad/neurocases/Neurocase01.htm. Retrieved 2008-02-06.
- ^ Gliomatosis Cerebri - My Child Has - Children's Hospital Boston
Nervous tissue tumors/NS neoplasm/Neuroectodermal tumor (ICD-O 9350–9589) (C70–C72, D32–D33, 191–192/225)
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Endocrine/
sellar (9350–9379) |
sellar: Craniopharyngioma · Pituicytoma
other: Pinealoma
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CNS
(9380–9539) |
Neuroepithelial
(brain tumors,
spinal tumors)
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Glioma
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Astrocyte
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Astrocytoma (Pilocytic astrocytoma, Pleomorphic xanthoastrocytoma, Fibrillary (also diffuse or lowgrade) astrocytomas, Anaplastic astrocytoma, Glioblastoma multiforme)
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Oligodendrocyte
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Oligodendroglioma
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Ependyma
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Ependymoma · Subependymoma
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Choroid plexus
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Choroid plexus tumor (Choroid plexus papilloma, Choroid plexus carcinoma)
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Multiple/unknown
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Oligoastrocytoma · Gliomatosis cerebri · Gliosarcoma
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Mature
neuron
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Ganglioneuroma: Ganglioglioma · Retinoblastoma · Neurocytoma · Dysembryoplastic neuroepithelial tumour · Lhermitte-Duclos disease
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PNET
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Neuroblastoma (Esthesioneuroblastoma, Ganglioneuroblastoma) · Medulloblastoma · Atypical teratoid rhabdoid tumor
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Primitive
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Medulloepithelioma
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Meningiomas
(meninges)
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Meningioma, Hemangiopericytoma
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Hematopoietic
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Primary central nervous system lymphoma
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PNS: NST
(9540–9579) |
cranial and paraspinal nerves: Neurofibroma (Neurofibrosarcoma, Neurofibromatosis) · Neurilemmoma/Schwannoma (Acoustic neuroma) · Malignant peripheral nerve sheath tumor
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note: not all brain tumors are of nervous tissue, and not all nervous tissue tumors are in the brain (see brain metastases)
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anat(n/s/m/p/4/e/b/d/c/a/f/l/g)/phys/devp
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noco(m/d/e/h/v/s)/cong/tumr, sysi/epon, injr
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proc, drug(N1A/2AB/C/3/4/7A/B/C/D)
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anat(h/r/t/c/b/l/s/a)/phys(r)/devp/prot/nttr/nttm/ntrp
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noco/auto/cong/tumr, sysi/epon, injr
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UpToDate Contents
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Japanese Journal
- 2. 多房性嚢胞形成を伴うgliomatosis cerebriの1例(第43回群馬脳腫瘍研究会<一般演題1>)
- 本徳 浩二,曲澤 聡,石原 淳治,橋場 康弘,吉田 カツ江
- The Kitakanto medical journal 60(1), 87-87, 2010-02-01
- NAID 120002139554
Related Links
- 2009年11月8日 ... Gliomatosis cerebri 異形グリア細胞が腫瘤形成をせずに、びまん性に浸潤発育した状態 発症のピーク 40~50歳代、性差は無し臨床症状 性格変化、精神症状、頭痛、痙攣、錐 体路障害など多彩画像上の特徴 ①2つ以上の脳葉に進展し、通常は ...
Related Pictures
★リンクテーブル★
[★]
- 英
- intracranial hypertension, increased intracranial pressure
- 同
- 脳圧亢進 brain hypertension
- 関
- 頭蓋内圧、頭蓋内圧亢進症
PON.594-595
頭蓋内圧亢進を来す疾患
症状
- 頭痛、嘔吐、視力障害(うっ血乳頭)、外転神経麻痺(頭蓋内での走行が長いので障害を受けやすい?)
- 脳ヘルニアに至れば、意識障害、呼吸障害、クッシング現象(徐脈、血圧上昇)
- 頭痛は早朝に出現することが多い。血中CO2濃度が上昇にするため。(脳血管拡張にてさらなる脳圧の亢進が見られる?)
[★]
- 英
- astroblastoma、astrocytic glioma
- 同
- アストロブラストーマ
- 関
- 星細胞腫、星芽腫
- astrocytic glioma、gliomatosis cerebri、neuroepithelial neoplasm、neuroepithelial tumor、polar spongioblastoma
[★]
極性海綿芽腫、極性海綿芽細胞腫
- 関
- astroblastoma、gliomatosis cerebri、neuroepithelial neoplasm、neuroepithelial tumor
[★]
神経上皮腫瘍、神経上皮新生物
- 関
- astroblastoma、gliomatosis cerebri、neuroepithelial tumor、polar spongioblastoma
[★]
神経上皮腫瘍
- 関
- astroblastoma、gliomatosis cerebri、neuroepithelial neoplasm、polar spongioblastoma
[★]
大脳
- 関
- cerebral、cerebrum