MCID: PRV003
MIFTS: 42

Perivascular Epithelioid Cell Tumor malady

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Summaries for Perivascular Epithelioid Cell Tumor

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

MalaCards based summary: Perivascular Epithelioid Cell Tumor, also known as neoplasm with perivascular epithelioid cell differentiation, is related to lymphangioleiomyomatosis and tuberous sclerosis complex. 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 8 10
Neoplasm with Perivascular Epithelioid Cell Differentiation 8 62
 
Perivascular Epithelioid Cell Neoplasms 62
Pecoma 8


Classifications:

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


External Ids:

Disease Ontology8 DOID:2643
MeSH34 D054973
SNOMED-CT57 388601000
NCIt39 C38150

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/GeneDecks gene sharing:

(show top 50)    (show all 77)
idRelated DiseaseScoreTop Affiliating Genes
1lymphangioleiomyomatosis31.0DES, TSC2
2tuberous sclerosis complex30.9TSC2
3smooth muscle tumor30.7DES, ACTC1
4gastrointestinal stromal tumor30.7ACTC1, DES
5tuberous sclerosis30.5SYP, CCL26, TSC2
6leiomyosarcoma30.4DES, ACTC1
7angiomyolipoma30.4ACTC1, CCL26, TSC2, DES
8epithelioid leiomyosarcoma30.3DES, ACTC1
9leiomyoma30.2ACTC1, TSC2, DES
10fibromatosis30.2ACTC1, DES
11melanoma29.7ACTC1, TSC2, DES
12hepatitis10.7
13uterus perivascular epithelioid cell tumor10.6
14pancreatitis10.5
15fasciitis10.5ACTC1
16infantile myofibromatosis10.4DES
17acute lymphoblastic leukemia10.3
18leukemia10.3
19appendicitis10.3
20angiosarcoma10.3
21cervicitis10.3
22diverticulitis10.3
23lymphoblastic leukemia10.3
24neuroblastoma10.3
25prostatitis10.3
26myopericytoma10.3
27tubular adenocarcinoma10.2SYP, ACTC1
28pseudosarcomatous fibromatosis10.2DES, ACTC1
29spindle cell carcinoma10.2DES, ACTC1
30spindle cell sarcoma10.2DES, ACTC1
31desmoid tumor10.2ACTC1, 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 tumor10.2DES, 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:

32
Uterus, Liver, Colon, Cervix, Kidney, Lymph node, Lung, Pancreas, Bone, Heart, Smooth muscle, Skin, Prostate, 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 136)
idTitleAuthorsYear
1
Ectopic Prolactin Secretion From a Perivascular Epithelioid Cell Tumor (PEComa). (25127092)
2014
2
Fertility sparing treatment of a malignant uterine perivascular epithelioid cell tumor: A case report. (24567888)
2014
3
Malignant perivascular epithelioid cell tumor (PEComa) of cervix with TFE3 gene rearrangement: a case report. (25337301)
2014
4
Metastatic perivascular epithelioid cell tumor responding to mammalian target of rapamycin inhibition. (25006296)
2014
5
Perivascular epithelioid cell tumor (PEComa) with TFE3 gene rearrangement: Clinicopathological, immunohistochemical, and molecular features. (25239799)
2014
6
Neoadjuvant sirolimus for a large hepatic perivascular epithelioid cell tumor (PEComa). (24575738)
2014
7
Cutaneous metastasis of a perivascular epithelioid cell tumor. (24818188)
2014
8
A resected perivascular epithelioid cell tumor (PEComa) of the pancreas diagnosed using endoscopic ultrasound-guided fine-needle aspiration. (24042513)
2013
9
Perivascular epithelioid cell tumor of the liver: a case report and literature review. (23832736)
2013
10
MRI diagnosis of perivascular epithelioid cell tumor (PEComa) of the liver. (24068418)
2013
11
Pigmented perivascular epithelioid cell tumor of the liver: report of a rare case with brief review of literature. (23771383)
2013
12
Perivascular epithelioid cell tumor located retroperitoneally with pulmonary lymphangioleiomyomatosis: report of a case. (23515754)
2013
13
PEComa: A Perivascular Epithelioid Cell Tumor in the Liver-A Case Report and Review of the Literature. (24489554)
2013
14
A huge malignant perivascular epithelioid cell tumor (PEComa) of the uterine cervix and vagina. (24331571)
2013
15
Malignant perivascular epithelioid cell tumor of mesentery with lymph node involvement: a case report and review of literature. (23587410)
2013
16
An unusual ileum tumor in a young woman. Perivascular epithelioid cell tumor of gastrointestinal tract. (22546372)
2012
17
Perivascular epithelioid cell tumor of the sigmoid colon with transcription factor E3 expression. (21546294)
2012
18
Microscopic endometrial perivascular epithelioid cell nodules: a case report with the earliest presentation of a uterine perivascular epithelioid cell tumor. (22937790)
2012
19
Successful treatment with the mTOR inhibitor everolimus in a patient with perivascular epithelioid cell tumor. (22943457)
2012
20
Malignant perivascular epithelioid cell tumor of the mesentery: a case report and literature review. (22414975)
2012
21
Malignant perivascular epithelioid cell tumor of the esophagus. (22957287)
2012
22
Perivascular epithelioid cell tumor of the ileum presenting as diverticulitis. (22953133)
2012
23
Malignant perivascular epithelioid cell tumor of the uterus. (22532912)
2012
24
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
25
Sclerosing variant of perivascular epithelioid cell tumor in the female genital organs. (22126387)
2011
26
Perivascular epithelioid cell tumor of the ascending colon mesentery in a child: case report and review of the literature. (20385296)
2010
27
Fertility-sparing operation for recurrence of uterine cervical perivascular epithelioid cell tumor. (21139828)
2010
28
Perivascular epithelioid cell tumor of abdominal origin. (20471562)
2010
29
A perivascular epithelioid cell tumor of the stomach: an unsuspected diagnosis. (20101783)
2010
30
Clinical activity of mTOR inhibition with sirolimus in malignant perivascular epithelioid cell tumors: targeting the pathogenic activation of mTORC1 in tumors. (20048174)
2010
31
An unresectable malignant perivascular epithelioid cell tumor resistant to multiple combined chemotherapies: a unique case report. (20051914)
2010
32
Molecular pathology of lymphangioleiomyomatosis and other perivascular epithelioid cell tumors. (20073603)
2010
33
Perivascular epithelioid cell tumor (PEComa) of abdominal cavity from falciform ligament: a case report. (19399284)
2009
34
Perivascular epithelioid cell tumors of the laryngopharynx: three case reports and literature review. (19409723)
2009
35
Perivascular epithelioid cell tumor (PEComa) of the liver diagnosed by contrast-enhanced ultrasonography. (20009396)
2009
36
Malignant variant of sclerosing perivascular epithelioid cell tumor arising in the adnexa. (19851212)
2009
37
Perivascular epithelioid cell tumor. (19391667)
2009
38
Laparoscopic radical excision of primary round ligament perivascular epithelioid cell tumor mimicking leiomyoma. (19835808)
2009
39
Growing perivascular epithelioid cell tumor of the liver studied with contrast-enhanced ultrasonography and magnetic resonance imaging. (19023006)
2008
40
Perivascular epithelioid cell tumor of the liver coexisting with a gastrointestinal stromal tumor. (18205275)
2008
41
Primary perivascular epithelioid cell tumor of the liver not related to hepatic ligaments: hepatic PEComa as an emerging entity. (18712670)
2008
42
Pancreatic perivascular epithelioid cell tumor (PEComa)]. (18675170)
2008
43
Perivascular epithelioid cell tumor of the liver: a report of two cases and review of the literature. (17907305)
2007
44
Perivascular epithelioid cell tumor (PEComa) in the genitourinary tract. (17198309)
2007
45
Perivascular epithelioid cell tumor (PEComa) in the transverse colon of an adolescent: a case report. (17455882)
2007
46
Correction: Perivascular epithelioid cell tumor (PEComa) of the uterine cervix associated with intraabdominal "PEComatosis": A clinicopathological study with comparative genomic hybridization analysis. (15869711)
2005
47
Perivascular epithelioid cell tumor (PEComa) with perirenal manifestation. (15948750)
2005
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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Expression patterns in normal tissues for genes affiliated with Perivascular Epithelioid Cell Tumor

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Pathways for genes affiliated with Perivascular Epithelioid Cell Tumor

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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 cardiomyopathy37
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/GeneDecks:

(show all 27)
idCompoundScoreTop Affiliating Genes
1orcein449.7ACTC1, DES
2procollagen449.6ACTC1, DES
3ganglioside449.6DES, SYP
4biotin44 24 1111.5DES, ACTC1
5silver449.5DES, SYP
6imatinib44 50 1111.4ACTC1, DES
7gold449.4SYP, DES
8s 100449.3SYP, ACTC1, DES
9reticulin449.2DES, ACTC1, SYP
10mucicarmine449.2SYP, ACTC1, DES
11b 723449.2DES, ACTC1, SYP
12hematoxylin449.2DES, ACTC1, SYP
13formaldehyde44 2410.2SYP, ACTC1, DES
14creatinine449.1SYP, ACTC1, DES
15progesterone44 28 61 24 1113.1SYP, ACTC1, DES
16retinoic acid44 249.9DES, ACTC1, SYP
17cysteine448.7SYP, CCL26, DES
18calcium44 50 24 1111.5SYP, ACTC1, CCL26, DES
19glycogen44 249.4SYP, ACTC1, TSC2, DES
20paraffin448.4DES, TSC2, ACTC1, SYP
21testosterone44 61 24 1111.4SYP, ACTC1, TSC2, DES
22steroid448.4DES, TSC2, ACTC1, SYP
23vegf448.4DES, TSC2, ACTC1, SYP
24oxygen44 249.3DES, TSC2, CCL26, ACTC1
25estrogen448.3DES, TSC2, ACTC1, SYP
26tyrosine448.3DES, TSC2, ACTC1, SYP
27glucose448.3ACTC1, 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/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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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet