DiseaseID 13662
肾母细胞瘤易感性
disease
Hereditary leiomyomatosis and renal cell cancer (HLRCC) is a hereditary cancer syndrome characterized by a predisposition to cutaneous and uterine leiomyomas and, in some families, to renal cell cance
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Disease: 1Formula: 6Herb: 7Symptom: 12Target: 21Links: 49
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Record Fields
Scalar fields from the final disease record.
- Disease Id
- 13662
- Core Entity Id
- 71338
- Source Entity Count
- 1
- Preferred Name
- Wilms Tumor, Susceptibility To
- Name Cn
- 肾母细胞瘤易感性
- Name Pinyin
- Shen Mu Xi Bao Liu Yi Gan Xing
- Name En
- Wilms Tumor, Susceptibility To
- Name Latin
- Bilingual Status
- complete
- Disease Type
- disease
- Umls Disease Type
- Finding
- Disgenet Type
- disease
- Mesh Class
- Female Urogenital Diseases and Pregnancy Complications; Male Urogenital Diseases; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; NeoplasmsNeoplasmsSkin and Connective Tissue Diseases; Female Urogenital Diseases and Pregnancy Complications; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Neoplasms
- Do Class
- Hpo Class
- Neoplasm; Abnormality of the integument
- Mesh Class Name
- Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Neoplasms; Female Urogenital Diseases and Pregnancy Complications; Skin and Connective Tissue DiseasesNeoplasms
- Hpo Class Name
- Abnormality of the integument; Neoplasm
- Do Class Name
- Disease Definition
- Hereditary leiomyomatosis and renal cell cancer (HLRCC) is a hereditary cancer syndrome characterized by a predisposition to cutaneous and uterine leiomyomas and, in some families, to renal cell cance
- Version
- v1,v2
- Suppressed
- No
Names
Preferred names, aliases, and source labels retained in the final schema.
Name
Wilms Tumor, Susceptibility To
Role
preferred
Name
Hereditary Leiomyomatosis And Renal Cell Cancer
Role
preferred
Name
Wilms Tumor 1
Role
preferred
Name
Wilms Tumor And Radial Bilateral Aplasia
Role
preferred
Name
Leiomyomatosis And Renal Cell Cancer, Hereditary
Role
alias
Name
Multiple Cutaneous Leiomyomas
Role
alias
Name
Wilms Tumor 5
Role
alias
Name
Wilms Tumor 6
Role
alias
Cross References
Trusted external identifiers retained for this final record.
Hpo
HP:0007437
Herb
HBDIS014804HBDIS022881HBDIS028757
Me Sh
D0093
Omim
150800601583616806
Umls
C0027708C1708350
Icd10
C64
Med Dra
10029145
Sym Map
SMDE00259SMDE03830
Dis Ge Net
C1708350C1832099C3891301
Orphanet
523654
Umls Sty
T033T191
Hpo Class
HP:0001574HP:0002664
Me Sh Class
C04C12C13C16C17
Etcm Disease
Hereditary Leiomyomatosis and Renal Cell Cancer
Tcmbank Disease
2552730
Itcmdb Generated
ITX-DISEASE-BBFC213D01F5
Attributes
Merged source attributes and domain-specific metadata.
Version
v1,v2
Suppress
0
Page Title
Disease Hereditary Leiomyomatosis and Renal Cell Cancer Details page
Disease Type
diseasephenotype
Hpo Class Name
Abnormality of the integument; Neoplasm
Hpo Disease Class
Neoplasm; Abnormality of the integument
Umls Disease Type
FindingNeoplastic Process
Basic Information
Disease Name
Hereditary Leiomyomatosis and Renal Cell Cancer
Global Category
Cancer diseases;Genetic diseases;Rare diseases
Anatomical Category
Blood diseases;Nephrological diseases;Skin diseases
Disease Definition
Hereditary leiomyomatosis and renal cell cancer (HLRCC) is a hereditary cancer syndrome characterized by a predisposition to cutaneous and uterine leiomyomas and, in some families, to renal cell canceNCI2016_NICHD_1602D:A malignant neoplasm of embryonal origin arising within the kidney.|NCI2016_NCI-GLOSS_1602D:A disease in which malignant (cancer) cells are found in the kidney, and may spread to the lungs, liver, or nearby lymph nodes. Wilms tumor usually occurs in children younger than 5 years old.|NCI2016_CDISC_1602D:A malignant embryonal neoplasm of the kidney.|NCI2016_02D:An embryonal pediatric tumor of the kidney which may also be seen rarely in adults. The peak incidence of Wilms tumor is between the second and fifth year of life. Microscopically, it is composed of a mixture of cellular elements (blastemal, stromal, and epithelial). The most common sites of metastasis include the regional lymph nodes, lungs, and liver.|NCI2016_02D:An embryonal neoplasm characterized by the presence of epithelial, mesenchymal, and blastema components. The vast majority of cases arise from the kidney. A small number of cases with morphologic features resembling Wilms tumor of the kidney have been reported arising from the ovary and the cervix.|MSH2017_2016_08_12:A malignant kidney tumor, caused by the uncontrolled multiplication of renal stem (blastemal), stromal (STROMAL CELLS), and epithelial (EPITHELIAL CELLS) elements. However, not all three are present in every case. Several genes or chromosomal areas have been associated with Wilms tumor which is usually found in childhood as a firm lump in a child's side or ABDOMEN.|MEDLINEPLUS_20151021:<p>Wilms tumor is a rare type of kidney cancer. It causes a tumor on one or both kidneys. It usually affects children, but can happen in adults. Having certain genetic conditions or birth defects can increase the risk of getting it. Children that are at risk should be screened for Wilms tumor every three months until they turn eight.</p> <p>Symptoms include a lump in the abdomen, blood in the urine, and a fever for no reason. Tests that examine the kidney and blood are used to find the tumor.</p> <p>Doctors usually diagnose and remove the tumor in surgery. Other treatments include chemotherapy and radiation and biologic therapies. Biologic therapy boosts your body's own ability to fight cancer.</p> <p >NIH: National Cancer Institute</p>|HPO2016_07_04:The presence of a nephroblastoma, which is a neoplasm of the kidney that primarily affects children. [HPO:probinson]|CSP2006:pediatric nephroblastoma originating from embryonic kidney stem cells; usually affects children before the fifth year, but may occur in the fetus or rarely in later life; caused by defects in one or more tumor suppressor genes.|CSP2006:malignant kidney tumor made up of three cell types: blastemal, stromal, and epithelial, but not all present in every case.
Me Sh Disease Class
Female Urogenital Diseases and Pregnancy Complications; Male Urogenital Diseases; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; NeoplasmsNeoplasmsSkin and Connective Tissue Diseases; Female Urogenital Diseases and Pregnancy Complications; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Neoplasms
Dis Ge Net Disease Type
diseasephenotype
Disease Class Name Me Sh
Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Neoplasms; Female Urogenital Diseases and Pregnancy Complications; Skin and Connective Tissue DiseasesNeoplasms
Umls Semantic Type Name
FindingNeoplastic Process