ELST
MCID: END051
MIFTS: 26

Endolymphatic Sac Tumor (ELST)

Categories: Rare diseases

Aliases & Classifications for Endolymphatic Sac Tumor

MalaCards integrated aliases for Endolymphatic Sac Tumor:

Name: Endolymphatic Sac Tumor 20 17 70
Elst 20

Classifications:



External Ids:

UMLS 70 C2348239

Summaries for Endolymphatic Sac Tumor

MalaCards based summary : Endolymphatic Sac Tumor, also known as elst, is related to von hippel-lindau syndrome and retinal hemangioblastoma. An important gene associated with Endolymphatic Sac Tumor is VHL (Von Hippel-Lindau Tumor Suppressor), and among its related pathways/superpathways is Cytoskeleton remodeling Neurofilaments. Affiliated tissues include breast, bone and kidney, and related phenotypes are Decreased shRNA abundance (Z-score < -2) and Decreased shRNA abundance (Z-score < -2)

Wikipedia : 73 An endolymphatic sac tumor (ELST) is a very uncommon papillary epithelial neoplasm arising within the... more...

Related Diseases for Endolymphatic Sac Tumor

Diseases related to Endolymphatic Sac Tumor via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 112)
# Related Disease Score Top Affiliating Genes
1 von hippel-lindau syndrome 31.1 VHL LOC107303340
2 retinal hemangioblastoma 30.4 VHL LOC107303340
3 erythrocytosis, familial, 2 30.3 VHL LOC107303340
4 hemangioblastoma 30.2 VHL LOC107303340 KRT8
5 papilloma 30.2 KRT8 KRT7
6 renal cell carcinoma, nonpapillary 29.0 VHL LOC107303340 KRT8 KRT7
7 ataxia, combined cerebellar and peripheral, with hearing loss and diabetes mellitus 10.5
8 rare tumor 10.5
9 sensorineural hearing loss 10.5
10 paraganglioma 10.4
11 ataxia and polyneuropathy, adult-onset 10.3
12 branchiootic syndrome 1 10.3
13 facial paralysis 10.3
14 meniere disease 10.3
15 papilloma of choroid plexus 10.3
16 hydrocephalus 10.3
17 squamous cell papilloma 10.3
18 papillary adenoma 10.3
19 papillary adenocarcinoma 10.2
20 cerebellopontine angle tumor 10.2
21 congenital myasthenic syndrome with episodic apnea 10.1 VHL LOC107303340
22 angiomatosis 10.1 VHL LOC107303340
23 cerebellar angioblastoma 10.1 VHL LOC107303340
24 pheochromocytoma-paraganglioma 10.1 VHL LOC107303340
25 neurofibromatosis, type ii 10.0
26 otitis media 10.0
27 aneurysmal bone cysts 10.0
28 nasopharyngeal carcinoma 10.0
29 meningioma, familial 10.0
30 tumor predisposition syndrome 10.0
31 cutaneous telangiectasia and cancer syndrome, familial 10.0
32 fibrous dysplasia 10.0
33 b-lymphoblastic leukemia/lymphoma 10.0
34 intracranial meningioma 10.0
35 cholesteatoma of middle ear 10.0
36 acoustic neuroma 10.0
37 melanotic neuroectodermal tumor 10.0
38 glomus tumor 10.0
39 hemangioma 10.0
40 benign mesothelioma 10.0
41 capillary hemangioma 10.0
42 papillary carcinoma 10.0
43 neurilemmoma 10.0
44 atypical choroid plexus papilloma 10.0
45 cranial nerve palsy 10.0
46 clear cell renal cell carcinoma 10.0
47 secretory meningioma 10.0
48 lymphoplasmacyte-rich meningioma 10.0
49 benign ependymoma 10.0
50 rhabdoid meningioma 10.0

Graphical network of the top 20 diseases related to Endolymphatic Sac Tumor:



Diseases related to Endolymphatic Sac Tumor

Symptoms & Phenotypes for Endolymphatic Sac Tumor

GenomeRNAi Phenotypes related to Endolymphatic Sac Tumor according to GeneCards Suite gene sharing:

26 (show all 11)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased shRNA abundance (Z-score < -2) GR00366-A-6 9.43 KRT8
2 Decreased shRNA abundance (Z-score < -2) GR00366-A-71 9.43 KRT7 KRT8
3 Increased shRNA abundance (Z-score > 2) GR00366-A-126 9.4 KRT8
4 Increased shRNA abundance (Z-score > 2) GR00366-A-135 9.4 KRT7 KRT8
5 Increased shRNA abundance (Z-score > 2) GR00366-A-180 9.4 KRT8
6 Increased shRNA abundance (Z-score > 2) GR00366-A-192 9.4 KRT7 KRT8
7 Increased shRNA abundance (Z-score > 2) GR00366-A-40 9.4 KRT8
8 Increased shRNA abundance (Z-score > 2) GR00366-A-68 9.4 KRT7
9 Increased shRNA abundance (Z-score > 2) GR00366-A-72 9.4 KRT7
10 Increased shRNA abundance (Z-score > 2) GR00366-A-74 9.4 KRT7 KRT8
11 Increased shRNA abundance (Z-score > 2) GR00366-A-91 9.4 KRT7

Drugs & Therapeutics for Endolymphatic Sac Tumor

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Endolymphatic Sac Tumors in a Population of Patients With Von Hippel-Lindau Disease:The Natural History and Pathobiology, and a Prospective Non-Randomized Clinical Trial of Hearing Preservation Surgery in Patients With Early Stage Endolymphatic Sac Tumors Completed NCT00001668
2 Near-infrared Spectroscopy in Endovascular Treatment (Danish: Nær-infrarød Spektroskopi Ved endovaskulær Trombektomi) Completed NCT03738644
3 An International Collaborative Study: Screening for Endolymphatic Sac Tumours (ELSTs) in Von Hippel-Lindau (vHL) Patients Recruiting NCT02420067

Search NIH Clinical Center for Endolymphatic Sac Tumor

Genetic Tests for Endolymphatic Sac Tumor

Anatomical Context for Endolymphatic Sac Tumor

MalaCards organs/tissues related to Endolymphatic Sac Tumor:

40
Breast, Bone, Kidney, Uterus

Publications for Endolymphatic Sac Tumor

Articles related to Endolymphatic Sac Tumor:

(show top 50) (show all 199)
# Title Authors PMID Year
1
Reply to correspondence of Elst et al. 61
33548115 2021
2
Effect of fluid administration on scene to traffic accident patients by EMS personnel: a propensity score-matched study using population-based ambulance records and nationwide trauma registry in Japan. 61
33492423 2021
3
Delayed yet effective response of an endolymphatic sac tumor to radiosurgery: case report focusing on its radio-biological behavior. 61
33236933 2020
4
Feasibility study: spot-scanning proton arc therapy (SPArc) for left-sided whole breast radiotherapy. 61
33028378 2020
5
Long experience for the diagnosis and treatment of sporadic endolymphatic sac tumor in a single center. 61
32673897 2020
6
Papillary-cystic neoplasms of the middle ear are distinct from endolymphatic sac tumours. 61
32940914 2020
7
The endolymphatic sac tumor: challenges in the eradication of a localized disease. 61
32889625 2020
8
What is the driving force behind molecular triangles and their guests? A quantum chemical perspective about host-guest interactions. 61
32812576 2020
9
Improved outcomes for out-of-hospital cardiac arrest patients treated by emergency life-saving technicians compared with basic emergency medical technicians: A JCS-ReSS study report. 61
32422240 2020
10
68Ga-DOTATATE Uptake in an Endolymphatic Sac Tumor: Radiologic-Pathologic Correlation. 61
32433163 2020
11
Radiotherapy and Radiosurgery for Treatment of Residual or Recurrent Endolymphatic Sac Tumor Following Previous Microsurgical Resection. 61
32271262 2020
12
Clinical features and treatment of endolymphatic sac tumor. 61
32068496 2020
13
A novel energy layer optimization framework for spot-scanning proton arc therapy. 61
32040214 2020
14
[Endolymphatic sac tumor with symptoms of endolymphatic hydrops: a case report and reviews]. 61
32791624 2020
15
Clinical response to pazopanib in a patient with endolymphatic sac tumor not associated with von Hippel-Lindau syndrome. 61
32141326 2020
16
Transcanal endoscopic assisted skull base endolymphatic sac tumor resection: A rare disease with advanced technology. 61
32110234 2020
17
Fibrous dysplasia mimicking a pediatric endolymphatic sac tumor. 61
31770666 2020
18
Improve the dosimetric outcome in bilateral head and neck cancer (HNC) treatment using spot-scanning proton arc (SPArc) therapy: a feasibility study. 61
32000817 2020
19
Endolymphatic sac tumor: case report and literature review. 61
32922945 2020
20
von Hippel-Lindau Syndrome: Genetic Study of Case With a Rare Pathogenic Variant With Optic Nerve Hemangioblastoma, a Rare Phenotypic Expression. 61
32117777 2020
21
Melanotic Neuroectodermal Tumor Presenting as Endolymphatic Sac Tumor. 61
30955377 2019
22
"Endolymphatic sac tumour": A case report with review of literature. 61
31611452 2019
23
Clinicoradiologic characteristics of endolymphatic sac tumors. 61
31197530 2019
24
CAIX and pax-8 Commonly Immunoreactive in Endolymphatic Sac Tumors: A Clinicopathologic Study of 26 Cases with Differential Considerations for Metastatic Renal Cell Carcinoma in von Hippel-Lindau Patients. 61
30291511 2019
25
Endolymphatic sac tumor at the cerebellopontine angle: A case report and review of literature. 61
31051407 2019
26
Sequential Imaging in Patient With Suspected Menière's Disease Identifies Endolymphatic Sac Tumor. 61
30124615 2018
27
Expression of renal cell markers and detection of 3p loss links endolymphatic sac tumor to renal cell carcinoma and warrants careful evaluation to avoid diagnostic pitfalls. 61
30340515 2018
28
High-resolution MRI of the inner ear enables syndrome differentiation and specific treatment of cerebellar downbeat nystagmus and secondary endolymphatic hydrops in a postoperative ELST patient. 61
29644399 2018
29
Current Treatment of Endolymphatic Sac Tumor of the Temporal Bone. 61
29923043 2018
30
Case Series and Systematic Review of Radiation Outcomes for Endolymphatic Sac Tumors. 61
29738384 2018
31
Clinicopathological features of low-grade malignant endolymphatic sac tumors. 61
29482989 2018
32
Improve dosimetric outcome in stage III non-small-cell lung cancer treatment using spot-scanning proton arc (SPArc) therapy. 61
29486782 2018
33
Gamma knife radiosurgery in the management of endolymphatic sac tumors. 61
29497571 2018
34
[Tumors of the inner ear and adjacent structures]. 61
28875382 2017
35
Endolymphatic sac tumour in von Hippel-Lindau disease: management strategies. 61
29165437 2017
36
Post-surgery pCASL perfusion MRI of endolymphatic sac tumor. 61
28802960 2017
37
Sporadic Endolymphatic Sac Tumor-A Very Rare Cause of Hearing Loss, Tinnitus, and Dizziness. 61
28716765 2017
38
3D-guided direct puncture therapeutic embolization of intracranial tumors. 61
28251915 2017
39
Endolymphatic Sac Tumor in von Hippel-Lindau Disease: A Rare Case Report. 61
29204213 2017
40
Endolymphatic Sac Tumor in Two Dogs. 61
28171732 2017
41
Intraoperative Electrocochleography: A Window Into Endolymphatic Hydrops in a Patient With an Endolymphatic Sac Tumor. 61
28106624 2017
42
Plasmacytoma of the Temporal Bone, a Great Imitator: Report of Seven Cases and Comprehensive Review of the Literature. 61
28192381 2017
43
A blue middle ear mass: Cholesterol granuloma mimicking a glomus tumor and endolymphatic sac tumor. 61
27931523 2017
44
History of the Endolymphatic Sac: From Anatomy to Surgery. 61
27851655 2017
45
Three sporadic cases of endolymphatic sac tumor. 61
27841219 2016
46
Endolymphatic Sac Tumor Showing Increased Activity on 68Ga DOTATATE PET/CT. 61
27454593 2016
47
Giant Posterior Temporal Bone Arachnoid Granulations: CT and MRI Findings. 61
27295446 2016
48
Treatment of endolymphatic sac tumour (Papillary adenocarcinoma) of the temporal bone. 61
27330425 2016
49
Atypical radiographic features of skull base cholesterol granuloma. 61
26164292 2016
50
Characterization of endolymphatic sac tumors and von Hippel-Lindau disease in the International Endolymphatic Sac Tumor Registry. 61
25867206 2016

Variations for Endolymphatic Sac Tumor

Expression for Endolymphatic Sac Tumor

Search GEO for disease gene expression data for Endolymphatic Sac Tumor.

Pathways for Endolymphatic Sac Tumor

Pathways related to Endolymphatic Sac Tumor according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
10.66 KRT8 KRT7

GO Terms for Endolymphatic Sac Tumor

Cellular components related to Endolymphatic Sac Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 keratin filament GO:0045095 8.62 KRT8 KRT7

Biological processes related to Endolymphatic Sac Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cornification GO:0070268 8.62 KRT8 KRT7

Sources for Endolymphatic Sac Tumor

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10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
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29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
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44 MeSH
45 MESH via Orphanet
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49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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