central nervous system cavernous hemangioma

中枢神経系海綿状血管腫

WordNet

1. (physical chemistry) a sample of matter in which substances in different phases are in equilibrium; "in a static system oil cannot be replaced by water on a surface"; "a system generating hydrogen peroxide"
2. instrumentality that combines interrelated interacting artifacts designed to work as a coherent entity; "he bought a new stereo system"; "the system consists of a motor and a small computer"
3. a group of independent but interrelated elements comprising a unified whole; "a vast system of production and distribution and consumption keep the country going" (同)scheme
4. a complex of methods or rules governing behavior; "they have to operate under a system they oppose"; "that language has a complex system for indicating gender" (同)system of rules
5. a group of physiologically or anatomically related organs or parts; "the body has a system of organs for digestion"
6. a procedure or process for obtaining an objective; "they had to devise a system that did not depend on cooperation"
7. the living body considered as made up of interdependent components forming a unified whole; "exercise helped him get the alcohol out of his system"
8. in or near a center or constituting a center; the inner area; "a central position"
9. a workplace that serves as a telecommunications facility where lines from telephones can be connected together to permit communication (同)telephone exchange, exchange
10. easily agitated; "a nervous addict"; "a nervous thoroughbred"
11. of or relating to the nervous system; "nervous disease"; "neural disorder" (同)neural
12. being or suggesting a cavern; "vast cavernous chambers hollowed out of limestone"
13. benign angioma consisting of a mass of blood vessels; some appear as birthmarks (同)haemangioma

PrepTutorEJDIC

1. 〈C〉(関連した部分から成る)『体系』,系統,組織[網],装置 / 〈C〉(教育・政治などの)『制度』,機構;《the~》体制 / 〈C〉(思想・学問などの)『体系』,学説 / 〈C〉(…の)『方法』,方式,やり方《+of doing》 / 〈U〉正しい方針(筋道,順序) / 〈U〉《the~》(身体の)組織,系統 / 〈U〉《the~,one's~》身体,全身
2. 『中心の』,中央の,中心からの / 『主要な』,中心的な(main) / (音声が)中舌音の / 電話交換局(《英》[telephone]exchange)
3. 『神経の』 / 『神経質な』,神経過敏な / 『心配な』,不安な
4. ほら穴の多い / ほら穴のような

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/06/18 11:17:38」(JST)

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Central nervous system cavernous hemangioma
Classification and external resources
Histology of a cavernous hemangioma
ICD-10	D18 (ILDS D18.014)
ICD-9	228.0x
DiseasesDB	30031
eMedicine	radio/95
MeSH	D006392

Central nervous system cavernous hemangioma is a cavernous hemangioma that arises in the central nervous system (CNS). It can be considered to be a variant of hemangioma, and is characterized by grossly large dilated blood vessels and large vascular channels, less well circumscribed, and more involved with deep structures, with a single layer of endothelium and an absence of neuronal tissue within the lesions. These thinly walled vessels resemble sinusoidal cavities filled with stagnant blood. Blood vessels in patients with CCM can range from a few millimeters to several centimeters in diameter. Most lesions occur in the brain, but any organ may be involved.

Incidence

The incidence in the general population is roughly 0.5%, and clinical symptoms typically appear between 20 to 30 years of age. Once thought to be strictly congenital, these vascular lesions have been found to occur de novo. It may appear either sporadically or exhibit autosomal dominant inheritance.

Symptoms

Clinical symptoms of CNS origin include recurrent headaches, focal neurological deficits, hemorrhagic stroke, and seizures, but CCM can also be asymptomatic. The nature and severity of the symptoms depend on the lesion's location.

Diagnosis

Diagnosis is generally made by magnetic resonance imaging (MRI), particularly using a specific imaging technique known as a gradient-echo sequence MRI, which can unmask small or punctate lesions that may otherwise remain undetected. These lesions are also more conspicuous on FLAIR imaging compared to standard T2 weighing. FLAIR imaging is different from Gradient sequences, rather, it is similar to T2 weighing but suppresses free-flowing fluid signal. Sometimes quiescent CCMs can be revealed as incidental findings during MRI exams ordered for other reasons. Many cavernous hemangiomas are detected "accidentally" during MRIs searching for other pathologies. These "incidentalomas" are generally asymptomatic. In the case of hemorrhage, however, a CT scan is more efficient at showing new blood than an MRI, and when brain hemorrhage is suspected, a CT scan may be ordered first, followed by an MRI to confirm the type of lesion that has bled.

Sometimes the lesion appearance imaged by MRI remains inconclusive. Consequently neurosurgeons will order a cerebral angiogram or magnetic resonance angiogram (MRA). Since CCMs are low flow lesions (they are hooked into the venous side of the circulatory system), they will be angiographically occult (invisible). If a lesion is discernible via angiogram in the same location as in the MRI, then an arteriovenous malformation (AVM) becomes the primary concern.

CCMs & venous angiomas

DVA in MRI (T1 axial contrast enhanced)

In up to 30% there is a coincidence of CCM with a venous angioma, also known as a developmental venous anomaly (DVA). These lesions appear either as enhancing linear blood vessels or caput medusae, a radial orientation of small vessels that resemble the hair of Medusa from Greek mythology. These lesions are thought to represent developmental anomalies of normal venous drainage. These lesions should not be removed, as venous infarcts have been reported. When found in association with a CCM that needs resection, great care should be taken not to disrupt the angioma.

Familial forms

Familial forms of CCM occur at four known genetic loci. The gene for CCM1 encodes KRIT1 (krev interaction trapped 1), and has been found to bind to ICAP1alpha (integrin cytoplasmic domain associated protein alpha), a beta1 integrin associated protein. A particular mutation in CCM1 (the Q455X mutation), accounts for a cluster of cases in the Southwestern United States. This cluster, particularly in northern New Mexico, is an example of the founder effect; it has been traced back to early Spanish settlers.

The gene for CCM2 encodes a novel protein named malcavernin that contains a phosphotyrosine (PTB) binding domain. The exact biological function of CCM2 is not clear. Recently, it has been shown that CCM1 and CCM2 proteins as well as ICAP1alpha form a macromolecular complex in the cell. In addition, it appears that CCM2 protein may function as a scaffolding protein for MAP kinases that are essential in p38 activation responding to osmotic stress including MEKK3 and MKK3. It also binds to Rac and actin. Therefore, CCM2 protein is also called OSM (osmosensing scaffold for MEKK3).

The CCM3 gene is the most recently identified CCM gene . CCM3 is known as PDCD10 (programmed cell death 10), which was initially identified as a gene that is up-regulated during the induction of apoptosis (cell death) in TF-1, a human myeloid cell line. The precise role of the PDCD10 protein in the CCM pathway is not clear. It is recently shown that PDCD10 forms a complex with CCM1 protein (KRIT1) and CCM2 protein (OSM). PDCD10 interacts directly with OSM independent of KRIT1-OSM interaction. Research is ongoing to determine the function and properties of all three CCM gene products as well as the reaction pathways in which they are involved.

Evidence suggests that a fourth gene, CCM4, may also cause CCM, however it has yet to be identified.

Mutations in these genes account for 70 to 80 percent of all cases of cerebral cavernous malformations. The remaining 20 to 30 percent of cases may be due to other, still unidentified, genes.

References

External links

• Angioma Alliance, provides information and support for those affected
• Cavernoma Alliance UK - a charity created for and by people affected by cavernoma.
• International Cavernous Angioma Patient Registry
• Angioma Alliance Canada, provides information and support for those affected in Canada.

Malformation, Familial

• GeneReviews/NCBI/NIH/UW entry on Cerebral Cavernous
• Atlas of Pathology
• Brain Blood Vessel Disorder Help Site
• Incidental Findings with Brain MRI
• Imaging MR and CT of Cavernous Malformations

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

• 1. 中枢神経系の血管奇形 vascular malformations of the central nervous system
• 2. 肝血管腫 hepatic hemangioma
• 3. 小児における神経画像検査へのアプローチ approach to neuroimaging in children
• 4. 成人における播種性血管内凝固の臨床的特徴、診断、および治療 clinical features diagnosis and treatment of disseminated intravascular coagulation in adults
• 5. 脳アミロイドアンギオパチー cerebral amyloid angiopathy

English Journal

• Magnetic resonance imaging in childhood leukemia survivors treated with cranial radiotherapy: a cross sectional, single center study.

• Faraci M, Morana G, Bagnasco F, Barra S, Polo P, Hanau G, Fioredda F, Caruso S, Rossi A, Spaziante R, Haupt R.SourceHematology-Oncology Department, G. Gaslini Children's Research Institute, Genova, Italy. maurafaraci@ospedale-gaslini.ge.it
• Pediatric blood & cancer.Pediatr Blood Cancer.2011 Aug;57(2):240-6. doi: 10.1002/pbc.22923. Epub 2010 Dec 22.
• BACKGROUND: Children treated with cranial radiotherapy (CRT) for leukemia are at risk of developing central nervous system injuries. Magnetic resonance imaging (MRI) represents the examination method of choice for evaluating radiation-induced brain complications. The purpose of this report is to des
• PMID 21671360

• A rare case of extradural lumbar nerve root cavernoma.

• Petridis AK, Doukas A, Hugo HH, Barth H, Mehdorn HM.SourceDepartment of Neurosurgery, University Hospitals Schleswig-Holstein, Campus Kiel, Arnold-Heller Str. 3, 24105 Kiel, Germany. opticdisc@aol.com
• European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society.Eur Spine J.2011 Jul;20 Suppl 2:S348-9. Epub 2011 Apr 19.
• We present a very rare case of an extradural nerve root cavernoma of the lumbar spine. The patient had signs of radiculopathy for the last 3?years. Conservative treatment was unsuccessful. The MRI-image revealed a lesion mimicking neurinoma of the left L3 nerve root. Surgical removal of the lesion
• PMID 21503800

Japanese Journal

• Papillary glioneuronal tumor radiologically mimicking a cavernous hemangioma with hemorrhagic onset

• BUCCOLIERO Anna Maria,GIORDANO Flavio,MUSSA Federico,TADDEI Antonio,GENITORI Lorenzo,TADDEI Gian Luigi
• Neuropathology : official journal the Japanese Society of Neuropathology 26(3), 206-211, 2006-06-01
• NAID 10020386646

• A Case of Multiple Cavernous Hemangiomas with Skin and Central Nervous System Involvement

• EBARA Naoki,ASADA Hideo,MIYAGAWA Sachiko
• Journal of dermatology 31(10), 835-838, 2004-10-01
• NAID 10016259318

Related Links

• Cerebral cavernous malformation - Genetics Home Reference

Cerebral cavernous malformations (CCMs) are collections of small blood vessels (capillaries) in the brain that are enlarged and ... Central Nervous System Cavernous Hemangioma; Cerebral Cavernous Hemangioma; Familial Cavernous ...

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★リンクテーブル★

リンク元	「中枢神経系海綿状血管腫」
関連記事	「nervous」「central」「system」「cavernous」

「中枢神経系海綿状血管腫」

　　[★]

英

central nervous system cavernous hemangioma

「nervous」

　　[★]

• adj.

• 神経の、心配な、神経質な

関

anxiety、anxious、anxiously、care、concern、fear、nerve、nervi、nervousness、nervus、neural、neuro、neuron、neuroticism、trouble、uneasy、worrisome、worry

　 　 　

「central」

　　[★]

• adj.

• 中心の、中心的な、中心性の、中枢的な、中枢性の、中枢の、中枢神経性の

関

center、centrally、centrally acting、centre、centro、centrum、hub、medial、pivotal、pivotally

　 　 　

「system」

　　[★]

• n.

• 系、システム、体系、体制、方式

関

method、series、strain

　 　 　 　 　 　 　 　

「cavernous」

　　[★]

• adj.

• 空洞性の、(脳)海綿静脈洞の

関

cavitary