MCID: PRV003
MIFTS: 42

Perivascular Epithelioid Cell Tumor malady

Cancer diseases category
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Summaries for Perivascular Epithelioid Cell Tumor

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65Wikipedia, 33MalaCards
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Wikipedia:65 In oncology, PEComa, also PEC tumour and perivascular epithelioid cell tumour, is a family of... more...

MalaCards: Perivascular Epithelioid Cell Tumor, also known as neoplasm with perivascular epithelioid cell differentiation, is related to lymphangioleiomyomatosis and angiomyolipoma. 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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8Disease Ontology, 10DISEASES, 62UMLS, 35MeSH, 58SNOMED-CT, 40NCIt
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Classifications:

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


Aliases & Descriptions:

perivascular epithelioid cell tumor 8 10
neoplasm with perivascular epithelioid cell differentiation 8
perivascular epithelioid cell neoplasms 62
pecoma 8


External Ids:

Disease Ontology8 DOID:2643
MeSH35 D054973
SNOMED-CT58 388601000
NCIt40 C38150

Related Diseases for Perivascular Epithelioid Cell Tumor

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17GeneCards, 18GeneDecks
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Diseases related to Perivascular Epithelioid Cell Tumor via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 75)
idRelated DiseaseScoreTop Affiliating Genes
1lymphangioleiomyomatosis30.9DES, TSC2
2angiomyolipoma30.7ACTC1, CCL26, TSC2, DES
3smooth muscle tumor30.5DES, ACTC1
4gastrointestinal stromal tumor30.5ACTC1, DES
5tuberous sclerosis30.4SYP, CCL26, TSC2
6leiomyoma30.4ACTC1, TSC2, DES
7leiomyosarcoma30.2DES, ACTC1
8melanoma30.1ACTC1, TSC2, DES
9epithelioid leiomyosarcoma30.0DES, ACTC1
10fibromatosis30.0ACTC1, DES
11uterus perivascular epithelioid cell tumor10.5
12hepatitis10.5
13pancreatitis10.5
14appendicitis10.3
15angiosarcoma10.3
16cervicitis10.3
17diverticulitis10.3
18leukemia10.3
19lymphoblastic leukemia10.3
20neuroblastoma10.3
21prostatitis10.3
22myopericytoma10.3
23fasciitis10.1ACTC1
24infantile myofibromatosis10.1DES
25tuberous sclerosis complex10.1TSC2
26tubular adenocarcinoma10.0SYP, ACTC1
27pseudosarcomatous fibromatosis10.0DES, ACTC1
28spindle cell carcinoma10.0DES, ACTC1
29spindle cell sarcoma10.0DES, ACTC1
30desmoid tumor10.0ACTC1, DES
31cutaneous fibrous histiocytoma10.0DES, ACTC1
32neurofibroma10.0ACTC1, SYP
33adenosarcoma10.0DES, ACTC1
34dermatofibrosarcoma protuberans10.0DES, ACTC1
35fibrous histiocytoma10.0DES, ACTC1
36endometrial stromal sarcoma10.0ACTC1, DES
37hemangiopericytoma10.0DES, ACTC1
38fibroma10.0DES, ACTC1
39embryonal rhabdomyosarcoma10.0DES, ACTC1
40malignant mixed mullerian tumor10.0DES, ACTC1
41sertoli cell tumor10.0DES, SYP
42abrikosov's tumor10.0DES, ACTC1
43granular cell tumor10.0DES, SYP
44giant cell tumor10.0DES, ACTC1
45papillary carcinoma10.0SYP, ACTC1
46malignant fibroxanthoma10.0DES, ACTC1
47pleomorphic adenoma10.0DES, ACTC1
48carcinoid syndrome10.0SYP, ACTC1
49neurilemmoma10.0ACTC1, DES
50benign mesothelioma10.0ACTC1, 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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42NIH Clinical Center, 6ClinicalTrials
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Drug clinical trials:

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Genetic Tests for Perivascular Epithelioid Cell Tumor

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Anatomical Context for Perivascular Epithelioid Cell Tumor

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33MalaCards
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MalaCards organs/tissues related to Perivascular Epithelioid Cell Tumor:

33
Uterus, Liver, Colon, Kidney, Cervix, Pancreas, Lymph node, Lung, Prostate, Skin, Smooth muscle, Heart, 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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52PubMed
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Articles related to Perivascular Epithelioid Cell Tumor:

(show top 50)    (show all 117)
idTitleAuthorsYear
1
A resected perivascular epithelioid cell tumor (PEComa) of the pancreas diagnosed using endoscopic ultrasound-guided fine-needle aspiration. (24042513)
2013
2
Perivascular epithelioid cell tumor of the liver: a case report and literature review. (23832736)
2013
3
MRI diagnosis of perivascular epithelioid cell tumor (PEComa) of the liver. (24068418)
2013
4
Pigmented perivascular epithelioid cell tumor of the liver: report of a rare case with brief review of literature. (23771383)
2013
5
Perivascular epithelioid cell tumor located retroperitoneally with pulmonary lymphangioleiomyomatosis: report of a case. (23515754)
2013
6
A huge malignant perivascular epithelioid cell tumor (PEComa) of the uterine cervix and vagina. (24331571)
2013
7
Malignant perivascular epithelioid cell tumor of mesentery with lymph node involvement: a case report and review of literature. (23587410)
2013
8
Malignant perivascular epithelioid cell tumor of the retroperitoneum. (24133607)
2013
9
Transcription factor E3 protein-positive perivascular epithelioid cell tumor of the appendix presenting as acute appendicitis: a case report and review of the literature. (23451753)
2013
10
An unusual ileum tumor in a young woman. Perivascular epithelioid cell tumor of gastrointestinal tract. (22546372)
2012
11
Perivascular epithelioid cell tumor of the sigmoid colon with transcription factor E3 expression. (21546294)
2012
12
Microscopic endometrial perivascular epithelioid cell nodules: a case report with the earliest presentation of a uterine perivascular epithelioid cell tumor. (22937790)
2012
13
Successful treatment with the mTOR inhibitor everolimus in a patient with perivascular epithelioid cell tumor. (22943457)
2012
14
Malignant perivascular epithelioid cell tumor of the mesentery: a case report and literature review. (22414975)
2012
15
Malignant perivascular epithelioid cell tumor of the esophagus. (22957287)
2012
16
Perivascular epithelioid cell tumor of the ileum presenting as diverticulitis. (22953133)
2012
17
Malignant perivascular epithelioid cell tumor of the uterus. (22532912)
2012
18
Sinonasal perivascular epithelioid cell tumor: benign or malignant neoplasm? (22643949)
2012
19
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
20
Sclerosing variant of perivascular epithelioid cell tumor in the female genital organs. (22126387)
2011
21
Perivascular epithelioid cell tumor of the ascending colon mesentery in a child: case report and review of the literature. (20385296)
2010
22
Fertility-sparing operation for recurrence of uterine cervical perivascular epithelioid cell tumor. (21139828)
2010
23
Perivascular epithelioid cell tumor of abdominal origin. (20471562)
2010
24
A perivascular epithelioid cell tumor of the stomach: an unsuspected diagnosis. (20101783)
2010
25
Clinical activity of mTOR inhibition with sirolimus in malignant perivascular epithelioid cell tumors: targeting the pathogenic activation of mTORC1 in tumors. (20048174)
2010
26
An unresectable malignant perivascular epithelioid cell tumor resistant to multiple combined chemotherapies: a unique case report. (20051914)
2010
27
Molecular pathology of lymphangioleiomyomatosis and other perivascular epithelioid cell tumors. (20073603)
2010
28
Perivascular epithelioid cell tumor (PEComa) of abdominal cavity from falciform ligament: a case report. (19399284)
2009
29
Perivascular epithelioid cell tumor (PEComa) of the pancreas: immunoelectron microscopy and review of the literature. (19712133)
2009
30
Perivascular epithelioid cell tumors of the laryngopharynx: three case reports and literature review. (19409723)
2009
31
Perivascular epithelioid cell tumor (PEComa) of the liver diagnosed by contrast-enhanced ultrasonography. (20009396)
2009
32
Malignant variant of sclerosing perivascular epithelioid cell tumor arising in the adnexa. (19851212)
2009
33
Perivascular epithelioid cell tumor. (19391667)
2009
34
Laparoscopic radical excision of primary round ligament perivascular epithelioid cell tumor mimicking leiomyoma. (19835808)
2009
35
Pigmented perivascular epithelioid cell tumor of the kidney. (19961256)
2009
36
Growing perivascular epithelioid cell tumor of the liver studied with contrast-enhanced ultrasonography and magnetic resonance imaging. (19023006)
2008
37
Perivascular epithelioid cell tumor of the liver coexisting with a gastrointestinal stromal tumor. (18205275)
2008
38
Primary perivascular epithelioid cell tumor of the liver not related to hepatic ligaments: hepatic PEComa as an emerging entity. (18712670)
2008
39
Pancreatic perivascular epithelioid cell tumor (PEComa)]. (18675170)
2008
40
"Malignant" uterine perivascular epithelioid cell tumor, pelvic lymph node lymphangioleiomyomatosis, and gynecological pecomatosis in a patient with tuberous sclerosis: a case report and review of the literature. (18156981)
2008
41
Perivascular epithelioid cell tumor of the liver: a report of two cases and review of the literature. (17907305)
2007
42
Perivascular epithelioid cell tumor (PEComa) in the genitourinary tract. (17198309)
2007
43
Perivascular epithelioid cell tumor (PEComa) in the transverse colon of an adolescent: a case report. (17455882)
2007
44
Correction: Perivascular epithelioid cell tumor (PEComa) of the uterine cervix associated with intraabdominal "PEComatosis": A clinicopathological study with comparative genomic hybridization analysis. (15869711)
2005
45
Perivascular epithelioid cell tumor (PEComa) with perirenal manifestation. (15948750)
2005
46
Perivascular epithelioid cell tumor of the orbit: a case report and review of the literature. (15803216)
2005
47
Perivascular epithelioid cell tumor of the uterus: a case report. (15213607)
2004
48
Perivascular epithelioid cell tumor of the uterus: immunohistochemical, ultrastructural and molecular study. (14629307)
2003
49
Recurrent perivascular epithelioid cell tumor of the uterus (PEComa): an immunohistochemical study and review of the literature. (13678746)
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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2BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Perivascular Epithelioid Cell Tumor

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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50PathCards, 38NCBI BioSystems Database, 55Reactome, 30KEGG, 53QIAGEN
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Pathways related to Perivascular Epithelioid Cell Tumor according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.4ACTC1, DES
2
Show member pathways
Arrhythmogenic right ventricular cardiomyopathy38
9.4ACTC1, DES
3
Show member pathways
8.5CCL26, TSC2, DES

Compounds for genes affiliated with Perivascular Epithelioid Cell Tumor

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45Novoseek, 24HMDB, 11DrugBank, 51PharmGKB, 29IUPHAR, 61Tocris Bioscience
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Compounds related to Perivascular Epithelioid Cell Tumor according to GeneCards/GeneDecks:

(show all 27)
idCompoundScoreTop Affiliating Genes
1orcein459.7ACTC1, DES
2procollagen459.6ACTC1, DES
3ganglioside459.6DES, SYP
4biotin45 24 1111.5DES, ACTC1
5silver459.5DES, SYP
6imatinib45 51 1111.4ACTC1, DES
7gold459.4SYP, DES
8s 100459.3SYP, ACTC1, DES
9reticulin459.2DES, ACTC1, SYP
10mucicarmine459.2SYP, ACTC1, DES
11b 723459.2DES, ACTC1, SYP
12hematoxylin459.2DES, ACTC1, SYP
13formaldehyde45 2410.2SYP, ACTC1, DES
14creatinine459.1SYP, ACTC1, DES
15progesterone45 29 61 24 1113.1SYP, ACTC1, DES
16retinoic acid45 249.9DES, ACTC1, SYP
17cysteine458.7SYP, CCL26, DES
18calcium45 51 24 1111.5SYP, ACTC1, CCL26, DES
19glycogen45 249.4SYP, ACTC1, TSC2, DES
20paraffin458.4DES, TSC2, ACTC1, SYP
21testosterone45 61 24 1111.4SYP, ACTC1, TSC2, DES
22steroid458.4DES, TSC2, ACTC1, SYP
23vegf458.4DES, TSC2, ACTC1, SYP
24oxygen45 249.3DES, TSC2, CCL26, ACTC1
25estrogen458.3DES, TSC2, ACTC1, SYP
26tyrosine458.3DES, TSC2, ACTC1, SYP
27glucose458.3ACTC1, TSC2, DES

GO Terms for genes affiliated with Perivascular Epithelioid Cell Tumor

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16Gene Ontology
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Biological processes related to Perivascular Epithelioid Cell Tumor according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1endocytosisGO:0068979.1SYP, TSC2
2muscle filament slidingGO:0300499.1ACTC1, DES

Products for genes affiliated with Perivascular Epithelioid Cell Tumor

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Sources for Perivascular Epithelioid Cell Tumor

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4CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet