同: 血管芽腫

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/01/21 03:46:25」(JST)

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Hemangioblastomas (capilliary hemangioblastomas)^[1]^[2] are tumors of the central nervous system that originate from the vascular system usually during middle-age. Sometimes these tumors occur in other sites such as the spinal cord and retina.^[3] They may be associated with other diseases such as polycythemia (increased blood cell count), pancreatic cysts and Von Hippel-Lindau syndrome (VHL syndrome). Hemangioblastomas are most commonly composed of stromal cells in small blood vessels and usually occur in the cerebellum, brain stem or spinal cord. They are classed as grade one tumors under the World Health Organization's classification system.^[2]

Pathogenesis

Hemangioblastomas are composed of endothelial cells, pericytes and stromal cells. In VHL syndrome the von Hippel-Lindau protein (pVHL) is dysfunctional, usually due to mutation and/or gene silencing. In normal circumstances, pVHL is involved in the inhibition of hypoxia-inducible factor 1 α (HIF-1α) by ubiquitin mediated proteosomal degradation. In these dysfunctional cells pVHL cannot degrade HIF-1α, causing it to accumulate. HIF-1α causes the production of vascular endothelial growth factor, platelet derived growth factor B, erythropoietin and transforming growth factor alpha, which act to stimulate growth of cells within the tumor.^[4]

Diagnosis

Contrast enhanced MRI of the conus medullaris and cauda equina of patient with VHL depicts a hemangioblastoma of the conus with extension into the nerve roots.

The primary diagnosis is made with a computed tomography scan (CT scan). On a scan, hemangioblastoma shows as a well-defined, low attenuation region in the posterior fossa with an enhancing nodule on the wall. Sometimes multiple lesions are present.^[3]

Treatment

The treatment for hemangioblastoma is surgical excision of the tumor.^[5] Although usually straightforward to carry out, recurrence of the tumor or more tumors at a different site develop in approximately 20% of patients.^[3] Gamma Knife Radiosurgery as well as LINAC have also been employed to successfully treat recurrence and control tumor growth of cerebellar hemangioblastomas.

Prognosis

The outcome for hemangioblastoma is very good, if surgical extraction of the tumor can be achieved; excision is possible in most cases and permanent neurologic deficit is uncommon and can be avoided altogether if the tumor is diagnosed and treated early. Persons with VHL syndrome have a bleaker prognosis than those who have sporadic tumors since those with VHL syndrome usually have more than one lesion.^[2]

Complications

Hemangioblastomas can cause polycythemia due to ectopic production of erythropoietin as a paraneoplastic syndrome.

Epidemiology

Hemangioblastoma are the rarest central nervous system tumors, accounting for less than 2%. Hemangioblastomas usually occur in adults, yet tumors may appear in VHL syndrome at much younger ages. Men and women are approximately at the same risk. Although they can occur in any section of the central nervous system, they usually occur in either side of the cerebellum, the brain stem or the spinal cord.^[2]^[6]

Additional images

Micrograph of cerebellar hemangioblastoma. HPS stain.
Micrograph of cerebellar hemangioblastoma. HPS stain.

References

^ http://neurosurgery.mgh.harvard.edu/newwhobt.htm
^ ^a ^b ^c ^d Louis, David N (1991). WHO Classification of Tumors of the Central Nervous System. IARC. ISBN 92-832-2430-2.
^ ^a ^b ^c Lindsay, Kenneth W; Ian Bone; Robin Callander; J. van Gijn (1991). Neurology and Neurosurgery Illustrated. United States: Churchill Livingstone. ISBN 0-443-04345-0.
^ Kaelin, William G (2005). "von Hippel–Lindau-associated malignancies: Mechanisms and therapeutic opportunities". Drug Discovery Today: Disease Mechanisms 2 (2): 225–231. doi:10.1016/j.ddmec.2005.04.003.
^ http://www.youtube.com/watch?v=7-pzApsNVRo
^ Richard, S; Campello C; Taillandier L; Parker F; Resche F. (1998). J Intern Med 243 (6): 547–553. PMID 9681857. Missing or empty |title= (help)

External links

Stereotactic Radiosurgery for Hemangioblastomas of the Brain

UpToDate Contents

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1. 血管芽細胞腫 hemangioblastoma
2. フォンヒッペル・リンダウ病の臨床的特徴、診断、およびマネージメント clinical features diagnosis and management of von hippel lindau disease
3. 末梢神経腫瘍 peripheral nerve tumors
4. 赤血球増加症患者に対する診断的アプローチ diagnostic approach to the patient with polycythemia
5. フォンヒッペル・リンダウ病の分子生物学および病因 molecular biology and pathogenesis of von hippel lindau disease

English Journal

Spinal cord tumors: new views and future directions.

Mechtler LL, Nandigam K.SourceDent Imaging and Neuro-Oncology Center, Dent Neurologic Institute, 3980 Sheridan Drive, Buffalo, NY 14226, USA; Roswell Park Cancer Institute, Buffalo, NY, USA; American Society of Neuro-Imaging, Minneapolis, MN, USA. Electronic address: lmechtler@dentinstitute.com.
Neurologic clinics.Neurol Clin.2013 Feb;31(1):241-68. doi: 10.1016/j.ncl.2012.09.011.
Spinal cord tumors are uncommon neoplasms that, without treatment, can cause significant neurologic morbidity and mortality. The historic classification of spine tumors is based on the use of myelography with 3 main groups: (1) extramedullary extradural, (2) intradural extramedullary, and (3) intrad
PMID 23186903

Clinical features of patients bearing central nervous system hemangioblastoma in von Hippel-Lindau disease.

Kanno H, Kuratsu J, Nishikawa R, Mishima K, Natsume A, Wakabayashi T, Houkin K, Terasaka S, Shuin T.SourceDepartment of Neurosurgery, Yokohama City University School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan, hiroshikannomd@nifty.com.
Acta neurochirurgica.Acta Neurochir (Wien).2013 Jan;155(1):1-7. doi: 10.1007/s00701-012-1514-y. Epub 2012 Oct 19.
BACKGROUND: Central nervous system (CNS) hemangioblastoma (HB) is one of the most common manifestations in von Hippel-Lindau disease (VHL), but large-scale studies on clinical features of CNS HB in VHL are scarce.METHODS: On the basis of the results of a questionnaire, we collected data of VHL patie
PMID 23080552

Japanese Journal

術前に髄外髄内2つの腫瘍の隣接形態を把握できなかったために二期的手術を行った脊髄血管芽腫の1例 (日本脊椎・脊髄神経手術手技学会特集号) -- (ビデオ手術手技)

本田英一郎,大石豪,久原隆弘 [他]
Journal of spine research : official journal of the Japanese Society for Spine Surgery and Related Research 3(7), 1024-1028, 2012-07
NAID 40019398186

脊髄髄内血管芽細胞腫摘出術における軟膜と栄養血管処理の重要性

寳子丸稔,木原俊壱,小泉徹,川那辺吉文,上田茂雄,佐々木伸洋,下村隆敏,藤田智昭,深谷春介,小林環
脊髄外科 : 日本脊髄外科研究会機関誌 = Spinal surgery : official journal of the Japanese Society of Spinal Surgery 26(1), 39-44, 2012-04-25
NAID 10030126095

Related Pictures

★リンクテーブル★

リンク元	「脳腫瘍」「血管芽腫」「血管細胞芽腫」

「脳腫瘍」

Hemangioblastoma
	Micrograph of a cerebellar hemangioblastoma. HPS stain.
Classification and external resources
ICD-10	D48.1
ICD-O	M9161/1
DiseasesDB	31512
eMedicine	med/2991 radio/326
MeSH	D018325
	[edit on Wikidata]

　　[★]

英: brain tumor, cerebral tumor
同: 脳新生物 brain neoplasm
関: 頭蓋内腫瘍

概念

分類

原発性脳腫瘍

神経膠腫 glioma

星状細胞腫 astrocytoma

胚腫 germinoma

転移性脳腫瘍

疫学

発生率：10万人対10-15人。罹患率：10万人対46人　→

腫瘍別発生頻度

腫瘍別発生頻度		小児	成人
神経膠腫	33%	星状細胞腫	髄膜腫
髄膜腫	22%	髄芽腫	膠芽腫
下垂体腺腫	15%	頭蓋咽頭腫	下垂体腺腫
神経鞘腫	9%	胚細胞腫	神経鞘腫
頭蓋咽頭腫	5%	上衣腫	転移性脳腫瘍

YN.J.188

部位	種類	小児	成人
頭蓋骨	頭蓋骨腫瘍	○	○
大脳半球	神経膠腫		○
大脳半球	髄膜腫		○
松果体	胚細胞腫	○
小脳半球	星細胞腫	○
小脳半球	血管芽腫		○
小脳虫部	髄芽腫	○
第四脳室	上衣腫	○
鞍上部・視交叉部・下垂体部	頭蓋咽頭腫	○
	視神経膠腫	○
	胚細胞腫	○
	下垂体腺腫		○
	髄膜腫		○
小脳橋角部	聴神経鞘腫		○
脳幹部	神経膠腫	○	○

小児の脳腫瘍

SCN.173

腫瘍：星細胞腫、髄芽腫、頭蓋咽頭腫、胚細胞腫、上衣腫の順に多い。
部位：1歳まではテント上、2-7歳まではテント下、8-15歳まではテント上に多い。

SRA.163

	後発年齢	好発部位
小脳星細胞腫	5～10歳	小脳半球
髄芽腫	5～10歳(男児に多い)	小脳虫部から発生
頭蓋咽頭腫	10～15歳	トルコ鞍上部
上衣腫	10～15,30～40歳	第四脳室、側脳室
髄芽腫	10～30歳	松果体部、トルコ鞍上部
脳幹部膠腫	～15歳	橋
視神経膠腫	～15歳	視神経視交叉