DiseaseID 6280

血管母细胞瘤

disease

NCI2016_02D:A WHO grade I tumor of uncertain histogenesis which is composed of stromal cells and abundant capillaries. Approximately 25% of hemangioblastomas are associated with Von Hippel-Lindau disease, a disease inher

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Disease: 1Symptom: 11Target: 24Links: 35
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Record Fields

Scalar fields from the final disease record.

Disease Id
6280
Core Entity Id
62909
Source Entity Count
1
Preferred Name
Hemangioblastoma
Name Cn
血管母细胞瘤
Name Pinyin
Xue Guan Mu Xi Bao Liu
Name En
Hemangioblastoma
Name Latin
Bilingual Status
complete
Disease Type
disease
Umls Disease Type
Neoplastic Process
Disgenet Type
disease
Mesh Class
Neoplasms
Do Class
disease of anatomical entity; disease of cellular proliferation
Hpo Class
Neoplasm; Abnormality of the nervous system
Mesh Class Name
Neoplasms
Hpo Class Name
Abnormality of the nervous system; Neoplasm
Do Class Name
disease of anatomical entity; disease of cellular proliferation
Disease Definition
NCI2016_02D:A WHO grade I tumor of uncertain histogenesis which is composed of stromal cells and abundant capillaries. Approximately 25% of hemangioblastomas are associated with Von Hippel-Lindau disease, a disease inherited through an autosomal dominant trait and characterized by the development of hemangioblastomas of the central nervous system and retina, clear cell renal carcinoma, pheochromocytoma, and pancreatic and inner ear tumors. (Adapted from WHO)|MSH2017_2016_08_12:A benign tumor of the nervous system that may occur sporadically or in association with VON HIPPEL-LINDAU DISEASE. It accounts for approximately 2% of intracranial tumors, arising most frequently in the cerebellar hemispheres and vermis. Histologically, the tumors are composed of multiple capillary and sinusoidal channels lined with endothelial cells and clusters of lipid-laden pseudoxanthoma cells. Usually solitary, these tumors can be multiple and may also occur in the brain stem, spinal cord, retina, and supratentorial compartment. Cerebellar hemangioblastomas usually present in the third decade with INTRACRANIAL HYPERTENSION, and ataxia. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, pp2071-2)|HPO2016_07_04:A hemangioblastoma is a benign vascular neoplasm that arises almost exclusively in the central nervous system. Hemangioblastomas consist of a tightly packed cluster of small blood vessels forming a mass of up to 1 or 2 cm in diameter. [HPO:probinson]
Version
v2
Suppressed
No

Names

Preferred names, aliases, and source labels retained in the final schema.

Name
Hemangioblastoma
Role
preferred
Name
Multiple Hemangioblastomas
Role
preferred
Name
Haemangioblastoma
Role
alias

Cross References

Trusted external identifiers retained for this final record.

Hpo
HP:0010797
Herb
HBDIS004460HBDIS011420
Me Sh
D018325
Umls
C0206734
Sym Map
SMDE09273
Do Class
DOID:14566DOID:7
Dis Ge Net
C0206734C0751692
Umls Sty
T191
Hpo Class
HP:0000707HP:0002664
Me Sh Class
C04
Etcm Disease
Hemangioblastoma
Tcmbank Disease
1970020225
Itcmdb Generated
ITX-DISEASE-6E155B2B55DDITX-DISEASE-EB0CAA719BF8

Attributes

Merged source attributes and domain-specific metadata.

Version
v2
Suppress
0
Page Title
Disease Hemangioblastoma Details page
Do Class Name
disease of anatomical entity; disease of cellular proliferation
Disease Type
disease
Hpo Class Name
Abnormality of the nervous system; Neoplasm
Do Disease Class
disease of anatomical entity; disease of cellular proliferation
Hpo Disease Class
Neoplasm; Abnormality of the nervous system
Umls Disease Type
Neoplastic Process
Basic Information
Disease Name
Hemangioblastoma
Global Category
Cancer diseases;Rare diseases
Anatomical Category
Cardiovascular diseases;Eye diseases;Neuronal diseases
Disease Definition
NCI2016_02D:A WHO grade I tumor of uncertain histogenesis which is composed of stromal cells and abundant capillaries. Approximately 25% of hemangioblastomas are associated with Von Hippel-Lindau disease, a disease inherited through an autosomal dominant trait and characterized by the development of hemangioblastomas of the central nervous system and retina, clear cell renal carcinoma, pheochromocytoma, and pancreatic and inner ear tumors. (Adapted from WHO)|MSH2017_2016_08_12:A benign tumor of the nervous system that may occur sporadically or in association with VON HIPPEL-LINDAU DISEASE. It accounts for approximately 2% of intracranial tumors, arising most frequently in the cerebellar hemispheres and vermis. Histologically, the tumors are composed of multiple capillary and sinusoidal channels lined with endothelial cells and clusters of lipid-laden pseudoxanthoma cells. Usually solitary, these tumors can be multiple and may also occur in the brain stem, spinal cord, retina, and supratentorial compartment. Cerebellar hemangioblastomas usually present in the third decade with INTRACRANIAL HYPERTENSION, and ataxia. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, pp2071-2)|HPO2016_07_04:A hemangioblastoma is a benign vascular neoplasm that arises almost exclusively in the central nervous system. Hemangioblastomas consist of a tightly packed cluster of small blood vessels forming a mass of up to 1 or 2 cm in diameter. [HPO:probinson]
Me Sh Disease Class
Neoplasms
Dis Ge Net Disease Type
disease
Disease Class Name Me Sh
Neoplasms
Umls Semantic Type Name
Neoplastic Process