ELST
MCID: END051
MIFTS: 25

Endolymphatic Sac Tumor (ELST) malady

Categories: Rare diseases

Aliases & Classifications for Endolymphatic Sac Tumor

Aliases & Descriptions for Endolymphatic Sac Tumor:

Name: Endolymphatic Sac Tumor 50 69
Elst 50

Classifications:



Summaries for Endolymphatic Sac Tumor

MalaCards based summary : Endolymphatic Sac Tumor, also known as elst, is related to von hippel-lindau syndrome and proteus-like syndrome. An important gene associated with Endolymphatic Sac Tumor is KRT8 (Keratin 8), and among its related pathways/superpathways is Cytoskeleton remodeling Neurofilaments. Affiliated tissues include bone, and related phenotypes are Increased shRNA abundance (Z-score > 2) and renal/urinary system

Wikipedia : 71 An endolymphatic sac tumor 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 all 41)
id Related Disease Score Top Affiliating Genes
1 von hippel-lindau syndrome 11.4
2 proteus-like syndrome 10.1 KRT7 KRT8
3 bartholin's gland adenoid cystic carcinoma 10.1 KRT7 KRT8
4 prostate lymphoma 10.1 KRT7 KRT8
5 middle ear adenocarcinoma 10.1 KRT7 KRT8
6 duodenal obstruction 10.1 KRT7 KRT8
7 sarcomatoid mesothelioma 10.1 KRT7 KRT8
8 adenoid squamous cell carcinoma 10.1 KRT7 KRT8
9 sensory organ benign neoplasm 10.1 KRT7 KRT8
10 rectum lymphoma 10.1 KRT7 KRT8
11 nasal cavity adenocarcinoma 10.1 KRT7 KRT8
12 distal arthrogryposis 10.1 KRT7 KRT8
13 vaginal villous adenoma 10.1 KRT7 KRT8
14 mongolian spot 10.1 KRT7 KRT8
15 generalized eruptive keratoacanthoma 10.1 KRT7 VHL
16 nodular tenosynovitis 10.1 KRT7 KRT8
17 main bronchus cancer 10.1 KRT7 VHL
18 malignant adult ependymoma 10.0 KRT7 KRT8
19 intestinal benign neoplasm 10.0 KRT7 KRT8
20 chronic granulomatous disease 10.0 KRT7 KRT8
21 odontotrichoungual-digital-palmar syndrome 10.0 KRT7 KRT8
22 granular cell leiomyosarcoma 10.0 KRT8 VHL
23 amyloidosis, hereditary, transthyretin-related 10.0 KRT7 KRT8
24 dysembryoplastic neuroepithelial tumor 10.0 KRT7 KRT8
25 placenta praevia 10.0 KRT7 KRT8
26 orchitis 9.9 KRT7 KRT8
27 autonomic nervous system neoplasm 9.8 KRT7 KRT8
28 aneurysmal bone cysts 9.8
29 choroiditis 9.8
30 pharyngitis 9.8
31 glomus tumor 9.8
32 adenocarcinoma 9.8
33 papillary adenocarcinoma 9.8
34 papillary adenoma 9.8
35 cerebellopontine angle tumor 9.8
36 hemangioblastoma 9.8
37 adenoma 9.8
38 aneurysm 9.8
39 hypoxia 9.8
40 bernard-soulier syndrome, type c 9.8 KRT7 KRT8 VHL
41 envenomization by bothrops lanceolatus 9.5 AVPR2 KRT7 KRT8 VHL

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
id Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-126 9.7 KRT8
2 Increased shRNA abundance (Z-score > 2) GR00366-A-135 9.7 KRT7 KRT8
3 Increased shRNA abundance (Z-score > 2) GR00366-A-192 9.7 KRT7 KRT8
4 Increased shRNA abundance (Z-score > 2) GR00366-A-6 9.7 KRT8
5 Increased shRNA abundance (Z-score > 2) GR00366-A-74 9.7 KRT8 KRT7
6 Decreased shRNA abundance (Z-score < -2) GR00366-A-180 9.5 KRT8
7 Decreased shRNA abundance (Z-score < -2) GR00366-A-40 9.5 KRT8
8 Decreased shRNA abundance (Z-score < -2) GR00366-A-68 9.5 KRT7
9 Decreased shRNA abundance (Z-score < -2) GR00366-A-71 9.5 KRT7 KRT8
10 Decreased shRNA abundance (Z-score < -2) GR00366-A-72 9.5 KRT7
11 Decreased shRNA abundance (Z-score < -2) GR00366-A-91 9.5 KRT7

MGI Mouse Phenotypes related to Endolymphatic Sac Tumor:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 renal/urinary system MP:0005367 8.8 AVPR2 KRT7 VHL

Drugs & Therapeutics for Endolymphatic Sac Tumor

Interventional clinical trials:


id Name Status NCT ID Phase
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 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:

39
Bone

Publications for Endolymphatic Sac Tumor

Articles related to Endolymphatic Sac Tumor:

(show top 50) (show all 81)
id Title Authors Year
1
A blue middle ear mass: Cholesterol granuloma mimicking a glomus tumor and endolymphatic sac tumor. ( 27931523 )
2017
2
Three sporadic cases of endolymphatic sac tumor. ( 27841219 )
2016
3
An Unusual Outcome After Mastoidectomy. Endolymphatic sac tumor. ( 26633706 )
2016
4
Endolymphatic Sac Tumor Showing Increased Activity on 68Ga DOTATATE PET/CT. ( 27454593 )
2016
5
Endolymphatic sac tumor in association with von Hippel-Lindau syndrome. ( 26991214 )
2016
6
Clinicopathologic study of endolymphatic sac tumor (ELST) and differential diagnosis of papillary tumors located at the cerebellopontine angle. ( 25944396 )
2015
7
Characterization of endolymphatic sac tumors and von Hippel-Lindau disease in the international ELST registry. ( 25867206 )
2015
8
An endolymphatic sac tumor with imaging features of aneurysmal bone cysts: differential diagnostic considerations. ( 24881035 )
2014
9
Endolymphatic sac tumor with von Hippel-Lindau disease: report of a case with atypical pathology of endolymphatic sac tumor. ( 24966975 )
2014
10
Cerebrospinal fluid cytology of an endolymphatic sac tumor. ( 25354959 )
2014
11
Sporadic endolymphatic sac tumor--a diagnostic and therapeutic challenge. ( 24966979 )
2014
12
Imaging detection of endolymphatic sac tumor-associated hydrops. ( 23472846 )
2013
13
Surgical resection of endolymphatic sac tumors in von Hippel-Lindau disease: findings, results, and indications. ( 23070752 )
2013
14
The Gruppo Otologico experience of endolymphatic sac tumor. ( 22326122 )
2013
15
Endolymphatic sac tumor. ( 23599098 )
2013
16
von Hippel-Lindau disease: deafness due to a non-MRI-visible endolymphatic sac tumor despite targeted screening. ( 24003980 )
2013
17
Endolymphatic sac tumor and otalgia. ( 23543975 )
2013
18
Papillary endolymphatic sac tumor: Catastrophic presentation in a child. ( 24060358 )
2013
19
Sporadic endolymphatic sac tumor: its clinical, radiological, and histological features, management, and follow-up. ( 22367728 )
2013
20
Papillary endolymphatic sac tumor: a case report. ( 22953101 )
2012
21
Diagnostic value of EAAT-1 and Kir7.1 for distinguishing endolymphatic sac tumors from choroid plexus tumors. ( 22706862 )
2012
22
Endolymphatic sac tumor and angiomatous lesions of the nasal and pharyngeal mucosa as a first manifestation of von Hippel-Lindau disease. ( 22777797 )
2012
23
Endolymphatic sac tumor: case report and review of the literature. ( 22472343 )
2012
24
Surgical treatment of endolymphatic sac tumor. ( 22201305 )
2012
25
von Hippel-Lindau disease: surveillance strategy for endolymphatic sac tumors. ( 21912262 )
2011
26
Gamma knife treatment of an endolymphatic sac tumor: unique features of a case and review of the literature. ( 21891944 )
2011
27
Endolymphatic sac tumor (aggressive papillary tumor of middle ear and temporal bone): sine qua non radiology-pathology and the University of Texas MD Anderson Cancer Center experience. ( 21167761 )
2011
28
Conservative management of endolymphatic sac tumors in von Hippel-Lindau disease: case report. ( 21103895 )
2011
29
Flap reconstruction and hyperbaric oxygen therapy in the management of temporal bone osteoradionecrosis in an endolymphatic sac tumor: case report. ( 23984214 )
2011
30
Endolymphatic sac tumor with overexpression of V2 receptor mRNA and inner ear hydrops. ( 21574774 )
2011
31
Isolate vertigo crisis revealing an endolymphatic sac tumor. ( 20495761 )
2010
32
Endolymphatic sac tumor presenting with MAcniA"re's disease. ( 20493370 )
2010
33
Endolymphatic sac tumors in von Hippel-Lindau disease: report of three cases. ( 20351605 )
2010
34
Endolymphatic sac tumor with von Hippel-Lindau disease: report of a case with analysis of von Hippel-Lindau gene and review. ( 20850701 )
2010
35
Endolymphatic sac tumor and von Hippel-Lindau disease in a single family. ( 20069227 )
2009
36
Endolymphatic sac tumor of cerebellopontine angle. ( 19670571 )
2009
37
An unusual presentation and spread of an endolymphatic sac tumor. ( 18520592 )
2008
38
Surgery for endolymphatic sac tumor: whether and when to keep hearing? ( 19086306 )
2008
39
Giant intracranial endolymphatic sac tumor (ELST). Case presentation and histogenetic considerations. ( 18273509 )
2008
40
Endolymphatic sac tumor (aggressive papillary tumor of middle ear and temporal bone): report of two cases with analysis of the VHL gene. ( 18423895 )
2008
41
Cochlear implantation for hearing loss associated with bilateral endolymphatic sac tumors in von Hippel-Lindau disease. ( 17468670 )
2007
42
Spinal metastasis from endolymphatic sac tumor. ( 17416807 )
2007
43
Endolymphatic sac tumor demonstrated by intralabyrinthine hemorrhage. Case report. ( 17695400 )
2007
44
Re: Cochlear implantation for hearing loss associated with bilateral endolymphatic sac tumors in von Hippel-Lindau disease. ( 17728688 )
2007
45
Endolymphatic sac tumor: a report of 3 cases and discussion of management. ( 17315832 )
2007
46
Origin of endolymphatic sac tumor. ( 20614260 )
2007
47
Cochlear implantation in a bilateral endolymphatic sac tumor patient. A case report. ( 17825433 )
2007
48
Endolymphatic sac tumors in von Hippel-Lindau disease. ( 17875849 )
2007
49
Endolymphatic sac tumor located around semicircular canals. ( 16466878 )
2006
50
Metastatic squamous carcinoma with features typical of an endolymphatic sac tumor. ( 16691152 )
2006

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:

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

GO Terms for Endolymphatic Sac Tumor

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

id Name GO ID Score Top Affiliating Genes
1 intermediate filament GO:0005882 8.96 KRT7 KRT8
2 keratin filament GO:0045095 8.62 KRT7 KRT8

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

id Name GO ID Score Top Affiliating Genes
1 keratinization GO:0031424 8.96 KRT7 KRT8
2 cornification GO:0070268 8.62 KRT7 KRT8

Molecular functions related to Endolymphatic Sac Tumor according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 structural molecule activity GO:0005198 8.62 KRT7 KRT8

Sources for Endolymphatic Sac Tumor

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