MCID: PRV003
MIFTS: 34

Perivascular Epithelioid Cell Tumor malady

Cancer diseases category

Summaries for Perivascular Epithelioid Cell Tumor

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MalaCards based summary: Perivascular Epithelioid Cell Tumor, also known as neoplasm with perivascular epithelioid cell differentiation, is related to lymphangioleiomyomatosis and smooth muscle tumor. An important gene associated with Perivascular Epithelioid Cell Tumor is ACTC1 (actin, alpha, cardiac muscle 1), and among its related pathways are Striated Muscle Contraction and Hypertrophic cardiomyopathy (HCM). The compounds orcein and procollagen have been mentioned in the context of this disorder. Affiliated tissues include uterus, liver and colon.

Aliases & Classifications for Perivascular Epithelioid Cell Tumor

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Perivascular Epithelioid Cell Tumor, Aliases & Descriptions:

Name: Perivascular Epithelioid Cell Tumor 9 11
Neoplasm with Perivascular Epithelioid Cell Differentiation 9
 
Perivascular Epithelioid Cell Neoplasms 60
Pecoma 9


Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer diseases


External Ids:

Disease Ontology9 DOID:2643
NCIt38 C38150
SNOMED-CT55 388601000
MeSH33 D054973

Related Diseases for Perivascular Epithelioid Cell Tumor

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Diseases related to Perivascular Epithelioid Cell Tumor via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50)    (show all 76)
idRelated DiseaseScoreTop Affiliating Genes
1lymphangioleiomyomatosis31.0DES, TSC2
2smooth muscle tumor30.7DES, ACTC1
3gastrointestinal stromal tumor30.7ACTC1, DES
4tuberous sclerosis30.5SYP, CCL26, TSC2
5leiomyosarcoma30.4DES, ACTC1
6angiomyolipoma30.3ACTC1, CCL26, TSC2, DES
7epithelioid leiomyosarcoma30.3DES, ACTC1
8leiomyoma30.2ACTC1, TSC2, DES
9fibromatosis30.2ACTC1, DES
10adenocarcinoma29.9TSC2, CCL26, ACTC1, SYP
11melanoma29.7ACTC1, TSC2, DES
12hepatitis10.7
13uterus perivascular epithelioid cell tumor10.6
14pancreatitis10.5
15prostatitis10.5
16fasciitis10.4ACTC1
17infantile myofibromatosis10.4DES
18neuroblastoma10.3
19leukemia10.3
20appendicitis10.3
21angiosarcoma10.3
22cervicitis10.3
23diverticulitis10.3
24lymphoblastic leukemia10.3
25myopericytoma10.3
26tuberous sclerosis-110.3TSC2
27tubular adenocarcinoma10.2SYP, ACTC1
28pseudosarcomatous fibromatosis10.2DES, ACTC1
29spindle cell carcinoma10.2DES, ACTC1
30spindle cell sarcoma10.2DES, ACTC1
31desmoid disease, hereditary10.2ACTC1, DES
32cutaneous fibrous histiocytoma10.2DES, ACTC1
33neurofibroma10.2ACTC1, SYP
34adenosarcoma10.2DES, ACTC1
35dermatofibrosarcoma protuberans10.2DES, ACTC1
36fibrous histiocytoma10.2DES, ACTC1
37endometrial stromal sarcoma10.2ACTC1, DES
38hemangiopericytoma10.2DES, ACTC1
39fibroma10.2DES, ACTC1
40embryonal rhabdomyosarcoma10.2DES, ACTC1
41malignant mixed mullerian tumor10.1DES, ACTC1
42sertoli cell tumor10.1DES, SYP
43abrikosov's tumor10.1DES, ACTC1
44granular cell tumor10.1DES, SYP
45giant cell tumor10.1DES, ACTC1
46papillary carcinoma10.1SYP, ACTC1
47malignant fibroxanthoma10.1DES, ACTC1
48pleomorphic adenoma10.1DES, ACTC1
49carcinoid syndrome10.1SYP, ACTC1
50neurilemmoma10.1ACTC1, DES

Graphical network of the top 20 diseases related to Perivascular Epithelioid Cell Tumor:



Diseases related to perivascular epithelioid cell tumor

Symptoms for Perivascular Epithelioid Cell Tumor

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Drugs & Therapeutics for Perivascular Epithelioid Cell Tumor

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Drug clinical trials:

Search ClinicalTrials for Perivascular Epithelioid Cell Tumor

Search NIH Clinical Center for Perivascular Epithelioid Cell Tumor

Genetic Tests for Perivascular Epithelioid Cell Tumor

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Anatomical Context for Perivascular Epithelioid Cell Tumor

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MalaCards organs/tissues related to Perivascular Epithelioid Cell Tumor:

31
Uterus, Liver, Colon, Cervix, Kidney, Lung, Pancreas, Lymph node, Prostate, Bone, Heart, Smooth muscle, Adrenal gland, Skin, Appendix

Animal Models for Perivascular Epithelioid Cell Tumor or affiliated genes

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Publications for Perivascular Epithelioid Cell Tumor

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Articles related to Perivascular Epithelioid Cell Tumor:

(show top 50)    (show all 142)
idTitleAuthorsYear
1
Perivascular epithelioid cell tumor of gastrointestinal tract: case report and review of the literature. (25621681)
2015
2
Hepatic perivascular epithelioid cell tumor: five case reports and literature review. (25554668)
2015
3
Ectopic Prolactin Secretion From a Perivascular Epithelioid Cell Tumor (PEComa). (25127092)
2014
4
Fertility sparing treatment of a malignant uterine perivascular epithelioid cell tumor: A case report. (24567888)
2014
5
Malignant perivascular epithelioid cell tumor (PEComa) of cervix with TFE3 gene rearrangement: a case report. (25337301)
2014
6
Metastatic perivascular epithelioid cell tumor responding to mammalian target of rapamycin inhibition. (25006296)
2014
7
Perivascular epithelioid cell tumor (PEComa) with TFE3 gene rearrangement: Clinicopathological, immunohistochemical, and molecular features. (25239799)
2014
8
Neoadjuvant sirolimus for a large hepatic perivascular epithelioid cell tumor (PEComa). (24575738)
2014
9
Cutaneous metastasis of a perivascular epithelioid cell tumor. (24818188)
2014
10
A resected perivascular epithelioid cell tumor (PEComa) of the pancreas diagnosed using endoscopic ultrasound-guided fine-needle aspiration. (24042513)
2013
11
Perivascular epithelioid cell tumor of the liver: a case report and literature review. (23832736)
2013
12
MRI diagnosis of perivascular epithelioid cell tumor (PEComa) of the liver. (24068418)
2013
13
Pigmented perivascular epithelioid cell tumor of the liver: report of a rare case with brief review of literature. (23771383)
2013
14
Perivascular epithelioid cell tumor located retroperitoneally with pulmonary lymphangioleiomyomatosis: report of a case. (23515754)
2013
15
PEComa: A Perivascular Epithelioid Cell Tumor in the Liver-A Case Report and Review of the Literature. (24489554)
2013
16
A huge malignant perivascular epithelioid cell tumor (PEComa) of the uterine cervix and vagina. (24331571)
2013
17
Malignant perivascular epithelioid cell tumor of mesentery with lymph node involvement: a case report and review of literature. (23587410)
2013
18
An unusual ileum tumor in a young woman. Perivascular epithelioid cell tumor of gastrointestinal tract. (22546372)
2012
19
Perivascular epithelioid cell tumor of the sigmoid colon with transcription factor E3 expression. (21546294)
2012
20
Microscopic endometrial perivascular epithelioid cell nodules: a case report with the earliest presentation of a uterine perivascular epithelioid cell tumor. (22937790)
2012
21
Successful treatment with the mTOR inhibitor everolimus in a patient with perivascular epithelioid cell tumor. (22943457)
2012
22
Malignant perivascular epithelioid cell tumor of the mesentery: a case report and literature review. (22414975)
2012
23
Malignant perivascular epithelioid cell tumor of the esophagus. (22957287)
2012
24
Perivascular epithelioid cell tumor of the ileum presenting as diverticulitis. (22953133)
2012
25
Malignant perivascular epithelioid cell tumor of the uterus. (22532912)
2012
26
Malignant perivascular epithelioid cell tumor (PEComa) of the uterus: serial imaging with F-18 FDG PET/CT for surveillance of recurrence and evaluation of response to therapy. (21368597)
2011
27
Sclerosing variant of perivascular epithelioid cell tumor in the female genital organs. (22126387)
2011
28
Perivascular epithelioid cell tumor of the ascending colon mesentery in a child: case report and review of the literature. (20385296)
2010
29
Fertility-sparing operation for recurrence of uterine cervical perivascular epithelioid cell tumor. (21139828)
2010
30
Perivascular epithelioid cell tumor of abdominal origin. (20471562)
2010
31
A perivascular epithelioid cell tumor of the stomach: an unsuspected diagnosis. (20101783)
2010
32
Clinical activity of mTOR inhibition with sirolimus in malignant perivascular epithelioid cell tumors: targeting the pathogenic activation of mTORC1 in tumors. (20048174)
2010
33
An unresectable malignant perivascular epithelioid cell tumor resistant to multiple combined chemotherapies: a unique case report. (20051914)
2010
34
Molecular pathology of lymphangioleiomyomatosis and other perivascular epithelioid cell tumors. (20073603)
2010
35
Perivascular epithelioid cell tumor (PEComa) of abdominal cavity from falciform ligament: a case report. (19399284)
2009
36
Perivascular epithelioid cell tumor (PEComa) of the pancreas: immunoelectron microscopy and review of the literature. (19712133)
2009
37
Perivascular epithelioid cell tumors of the laryngopharynx: three case reports and literature review. (19409723)
2009
38
Perivascular epithelioid cell tumor (PEComa) of the liver diagnosed by contrast-enhanced ultrasonography. (20009396)
2009
39
Malignant variant of sclerosing perivascular epithelioid cell tumor arising in the adnexa. (19851212)
2009
40
Growing perivascular epithelioid cell tumor of the liver studied with contrast-enhanced ultrasonography and magnetic resonance imaging. (19023006)
2008
41
Perivascular epithelioid cell tumor of the liver coexisting with a gastrointestinal stromal tumor. (18205275)
2008
42
Primary perivascular epithelioid cell tumor of the liver not related to hepatic ligaments: hepatic PEComa as an emerging entity. (18712670)
2008
43
Pancreatic perivascular epithelioid cell tumor (PEComa)]. (18675170)
2008
44
Perivascular epithelioid cell tumor of the liver: a report of two cases and review of the literature. (17907305)
2007
45
Perivascular epithelioid cell tumor (PEComa) in the genitourinary tract. (17198309)
2007
46
Perivascular epithelioid cell tumor (PEComa) in the transverse colon of an adolescent: a case report. (17455882)
2007
47
Correction: Perivascular epithelioid cell tumor (PEComa) of the uterine cervix associated with intraabdominal "PEComatosis": A clinicopathological study with comparative genomic hybridization analysis. (15869711)
2005
48
Perivascular epithelioid cell tumor (PEComa) with perirenal manifestation. (15948750)
2005
49
Perivascular epithelioid cell tumor of the uterus: immunohistochemical, ultrastructural and molecular study. (14629307)
2003
50
Malignant perivascular epithelioid cell tumor involving the prostate. (12562263)
2003

Variations for Perivascular Epithelioid Cell Tumor

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Expression for genes affiliated with Perivascular Epithelioid Cell Tumor

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Search GEO for disease gene expression data for Perivascular Epithelioid Cell Tumor.

Pathways for genes affiliated with Perivascular Epithelioid Cell Tumor

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Pathways related to Perivascular Epithelioid Cell Tumor according to GeneCards Suite gene sharing:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.4ACTC1, DES
2
Show member pathways
Arrhythmogenic right ventricular cardiomyopathy36
9.4ACTC1, DES
3
Show member pathways
8.5CCL26, TSC2, DES

Compounds for genes affiliated with Perivascular Epithelioid Cell Tumor

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Compounds related to Perivascular Epithelioid Cell Tumor according to GeneCards Suite gene sharing:

(show all 27)
idCompoundScoreTop Affiliating Genes
1orcein439.7ACTC1, DES
2procollagen439.6ACTC1, DES
3ganglioside439.6DES, SYP
4biotin43 24 1211.5DES, ACTC1
5silver439.5DES, SYP
6imatinib43 49 1211.4ACTC1, DES
7gold439.4SYP, DES
8s 100439.3SYP, ACTC1, DES
9reticulin439.2DES, ACTC1, SYP
10mucicarmine439.2SYP, ACTC1, DES
11b 723439.2DES, ACTC1, SYP
12hematoxylin439.2DES, ACTC1, SYP
13formaldehyde43 2410.2SYP, ACTC1, DES
14creatinine439.1SYP, ACTC1, DES
15progesterone43 28 59 24 1213.1SYP, ACTC1, DES
16retinoic acid43 249.9DES, ACTC1, SYP
17cysteine438.7SYP, CCL26, DES
18calcium43 49 24 1211.5SYP, ACTC1, CCL26, DES
19glycogen43 249.4SYP, ACTC1, TSC2, DES
20paraffin438.4DES, TSC2, ACTC1, SYP
21testosterone43 59 24 1211.4SYP, ACTC1, TSC2, DES
22steroid438.4DES, TSC2, ACTC1, SYP
23vegf438.4DES, TSC2, ACTC1, SYP
24oxygen43 249.3DES, TSC2, CCL26, ACTC1
25estrogen438.3DES, TSC2, ACTC1, SYP
26tyrosine438.3DES, TSC2, ACTC1, SYP
27glucose438.3ACTC1, TSC2, DES

GO Terms for genes affiliated with Perivascular Epithelioid Cell Tumor

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Biological processes related to Perivascular Epithelioid Cell Tumor according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1endocytosisGO:00068979.1SYP, TSC2
2muscle filament slidingGO:00300499.1ACTC1, DES

Products for genes affiliated with Perivascular Epithelioid Cell Tumor

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Sources for Perivascular Epithelioid Cell Tumor

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet