MCID: PRV023
MIFTS: 20

Perivascular Epithelioid Cell Neoplasm

Categories: Cancer diseases, Rare diseases

Aliases & Classifications for Perivascular Epithelioid Cell Neoplasm

MalaCards integrated aliases for Perivascular Epithelioid Cell Neoplasm:

Name: Perivascular Epithelioid Cell Neoplasm 58
Perivascular Epithelioid Tumour 58
Pecoma 58

Classifications:



External Ids:

Orphanet 58 ORPHA595133

Summaries for Perivascular Epithelioid Cell Neoplasm

MalaCards based summary : Perivascular Epithelioid Cell Neoplasm, also known as perivascular epithelioid tumour, is related to uterus perivascular epithelioid cell tumor and perivascular epithelioid cell tumor. An important gene associated with Perivascular Epithelioid Cell Neoplasm is TFE3 (Transcription Factor Binding To IGHM Enhancer 3). The drugs Sirolimus and Propranolol have been mentioned in the context of this disorder. Affiliated tissues include colon, pancreas and uterus.

Related Diseases for Perivascular Epithelioid Cell Neoplasm

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

(show top 50) (show all 102)
# Related Disease Score Top Affiliating Genes
1 uterus perivascular epithelioid cell tumor 11.1
2 perivascular epithelioid cell tumor 10.6
3 tuberous sclerosis 10.5
4 mesenchymal cell neoplasm 10.5
5 tuberous sclerosis 1 10.4
6 rare tumor 10.3
7 angiomyolipoma 10.3
8 melanoma 10.2
9 leiomyosarcoma 10.2
10 clear cell sarcoma 10.2
11 renal cell carcinoma, nonpapillary 10.1
12 alveolar soft part sarcoma 10.1
13 kidney angiomyolipoma 10.1
14 soft tissue sarcoma 10.1
15 adrenocortical carcinoma, hereditary 10.1
16 adenomyosis 10.1
17 lymphangioleiomyomatosis 10.1
18 adrenal cortical carcinoma 10.1
19 microphthalmia 10.1
20 leiomyoma 10.1
21 pineoblastoma 10.1
22 colonic benign neoplasm 10.1
23 granular cell tumor 10.1
24 hemangioma 10.1
25 hemangioma of liver 10.1
26 endometriosis 10.1
27 adrenal cortical adenocarcinoma 10.1
28 endometrial stromal sarcoma 10.1
29 uterine sarcoma 10.1
30 rapidly involuting congenital hemangioma 10.1
31 rare soft tissue tumor 10.1
32 paraganglioma 10.1
33 sarcoma 10.1
34 neuroendocrine tumor 10.0
35 spindle cell sarcoma 10.0
36 li-fraumeni syndrome 9.9
37 li-fraumeni syndrome 2 9.9
38 fibroma 9.9
39 amenorrhea 9.9
40 rhabdomyosarcoma 9.9
41 clear cell renal cell carcinoma 9.9
42 epithelioid leiomyosarcoma 9.9
43 posttransplant acute limbic encephalitis 9.9
44 cholangiocarcinoma 9.8
45 cardiac tamponade 9.8
46 pneumothorax 9.8
47 constipation 9.8
48 thrombocytosis 9.8
49 myoma 9.8
50 gallbladder cancer 9.8

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



Diseases related to Perivascular Epithelioid Cell Neoplasm

Symptoms & Phenotypes for Perivascular Epithelioid Cell Neoplasm

Drugs & Therapeutics for Perivascular Epithelioid Cell Neoplasm

Drugs for Perivascular Epithelioid Cell Neoplasm (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 11)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Sirolimus Approved, Investigational Phase 2 53123-88-9 5284616 6436030
2
Propranolol Approved, Investigational 525-66-6 4946
3
Miconazole Approved, Investigational, Vet_approved 22916-47-8 4189
4
Clotrimazole Approved, Vet_approved 23593-75-1 2812
5 Adrenergic beta-Antagonists
6 Antifungal Agents
7 Immunosuppressive Agents
8 Antibiotics, Antitubercular
9 Anti-Infective Agents
10 Immunologic Factors
11 Anti-Bacterial Agents

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Phase 2 Multi-center Investigation of Efficacy of ABI-009 (Nab-sirolimus) in Patients With Advanced Malignant Perivascular Epithelioid Cell Tumors (PEComa) Active, not recruiting NCT02494570 Phase 2 ABI-009
2 A Phase II Study of Orally Administered BEZ235 Monotherapy in Patients With Metastatic or Unresectable Malignant PEComa Withdrawn NCT01690871 Phase 2 BEZ235
3 Evaluation of New Biomarkers Predictive of Efficacy Betablockers in PEComa and Vascular Pediatric Tumors Completed NCT02334930
4 National Multicenter Retrospective Study: PEComas: Natural History and Prognostic Recruiting NCT03916575
5 Expanded Access for an Intermediate-size Population for ABI-009 (Sirolimus Albumin-bound Nanoparticles for Injectable Suspension) in Patients With Advanced Perivascular Epithelioid Cell Tumors (PEComa) and Patients With a Malignancy With Relevant Genetic Mutations or mTOR Pathway Activation Available NCT03817515 ABI-009

Search NIH Clinical Center for Perivascular Epithelioid Cell Neoplasm

Genetic Tests for Perivascular Epithelioid Cell Neoplasm

Anatomical Context for Perivascular Epithelioid Cell Neoplasm

MalaCards organs/tissues related to Perivascular Epithelioid Cell Neoplasm:

40
Colon, Pancreas, Uterus, Cervix

Publications for Perivascular Epithelioid Cell Neoplasm

Articles related to Perivascular Epithelioid Cell Neoplasm:

(show all 41)
# Title Authors PMID Year
1
Diagnostic dilemma for an adrenal mass: perivascular epithelioid cell neoplasm versus adrenocortical carcinoma. 61
33547853 2021
2
TFE3 Gene Rearrangement in Perivascular Epithelioid Cell Neoplasm (PEComa) of the Genitourinary Tract. 61
32576448 2020
3
Primary hepatic perivascular epithelioid cell neoplasm (PEComa) with fever in a 53-year-old man. 61
32051281 2020
4
Renal angiomyolipoma (AML) harboring a missense mutation of TSC2 with copy-neutral loss of heterozygosity (CN-LOH). 61
31847710 2020
5
Parietal Pleura-Based Malignant Perivascular Epithelioid Cell Neoplasm Protruding Into Serous Cavity: A Hitherto Unrecognized Occurrence. 61
31812753 2020
6
Endometrial polyp-like perivascular epithelioid cell neoplasm associated with TFE3 translocation: report of one case. 61
32269693 2020
7
Malignant Epithelioid Angiomyolipoma of the Kidney (Malignant Perivascular Epithelioid Cell Neoplasm). 61
31902422 2020
8
Feasibility of laparoscopic isolated caudate lobe resection for rare hepatic mesenchymal neoplasms. 61
31667169 2019
9
Rupture of perivascular epithelioid cell neoplasm at 34 weeks' gestation: A nonendometriosis case of spontaneous hemoperitoneum in pregnancy. 61
30548368 2019
10
[Clinical analysis of hepatic perivascular epithelioid cell neoplasm: a report of eleven cases]. 61
30220166 2018
11
Invagination as Manifestation of a Perivascular Epithelioid Cell Neoplasm (PEComa) of the Colon. 61
29915401 2018
12
Primary malignant perivascular epithelioid cell neoplasm (PEComa) of the bone mimicking granular cell tumor in core biopsy: A case report and literature review. 61
29435023 2018
13
TFE3-Expressing Epithelioid Rich Perivascular Epithelioid Cell Neoplasm (PEComa) of the Bladder with Unusual Benign Course. 61
29531006 2018
14
Hepatic perivascular epithelioid cell neoplasm: A clinical and pathological experience in diagnosis and treatment. 61
28413654 2017
15
TFE3-Expressing Perivascular Epithelioid Cell Neoplasm (PEComa) of the Sella Turcica. 61
27189222 2017
16
Malignant melanocytic neoplasm of pancreas with liver metastasis: Is it malignant melanoma or clear cell sarcoma? 61
28195103 2017
17
Malignant Perivascular Epithelioid Cell Neoplasm (PEComa) of the Pelvis: A Case Report. 61
27169023 2016
18
Hepatic multiple perivascular epithelioid cell neoplasm: A case report and literature review. 61
27073677 2016
19
Malignant TFE3-rearranged perivascular epithelioid cell neoplasm (PEComa) presenting as a subcutaneous mass. 61
26385848 2016
20
[Renal cell carcinoma with t(6;11)(p21.2;q13)/MALAT1-TFEB fusion: a clinical and pathological analysis]. 61
26888508 2015
21
Perivascular epithelioid cell neoplasm (PEComa) of the uterus: A systematic review. 61
25981307 2015
22
Primary retroperitoneal perivascular epithelioid cell neoplasm: A case report. 61
26171053 2015
23
Malignant perivascular epithelioid cell neoplasm of the mediastinum and the lung: one case report. 61
26039123 2015
24
TFE3 translocation-associated perivascular epithelioid cell neoplasm (PEComa) of the gynecologic tract: morphology, immunophenotype, differential diagnosis. 61
25517951 2015
25
Broad ligament perivascular epithelioid tumour presenting with haemoperitoneum. 61
25496346 2015
26
A 6-year-old girl with vaginal spotting who was diagnosed with perivascular epithelioid cell neoplasm after vaginoscopic resection. 61
25264534 2014
27
Perivascular epithelioid cell neoplasm (PEComa) of the gynecologic tract: clinicopathologic and immunohistochemical characterization of 16 cases. 61
24418852 2014
28
Malignant mesenteric perivascular epithelioid cell neoplasm presenting as an intra-abdominal fistula in a 49-year-old female. 61
25114821 2014
29
Malignant PEComa involving the mandible: report of a unique case. 61
22901659 2014
30
Malignant perivascular epithelioid cell neoplasm (PEComa) of the urinary bladder with TFE3 gene rearrangement: clinicopathologic, immunohistochemical, and molecular features. 61
23797724 2013
31
Perivascular epithelioid cell neoplasm of the urinary bladder in an adolescent: a case report and review of the literature. 61
23276164 2012
32
Primary perivascular epithelioid cell neoplasm (PEComa) of bone: report of two cases and review of the literature. 61
22752420 2012
33
Early onset of a nasal perivascular epithelioid cell neoplasm not related to tuberous sclerosis complex. 61
22767987 2012
34
"Malignant" perivascular epithelioid cell neoplasm: risk stratification and treatment strategies. 61
22619565 2012
35
Perivascular epithelioid cell neoplasm (PEComa) originating from the pancreas and metastasizing to the liver. 61
21926558 2011
36
CD1a immunopositivity in perivascular epithelioid cell neoplasms: true expression or technical artifact? A streptavidin-biotin and polymer-based detection system immunohistochemical study of perivascular epithelioid cell neoplasms and their morphologic mimics. 61
21194729 2011
37
Microscopic uterine lymphangioleiomyomatosis perivascular epithelioid cell neoplasm: a case report with the earliest manifestation of this enigmatic neoplasm. 61
21131829 2011
38
Perivascular epithelioid cell neoplasm of the uterine cervix: an unusual tumor in an unusual location. 61
21234248 2010
39
Perivascular epithelioid cell neoplasm of the colon. 61
21160599 2010
40
A heavily pigmented vaginal perivascular epithelioid cell neoplasm. 61
19757288 2009
41
Clear cell 'sugar' tumor (PEComa) of the skin: a case report. 61
15953380 2005

Variations for Perivascular Epithelioid Cell Neoplasm

Expression for Perivascular Epithelioid Cell Neoplasm

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

Pathways for Perivascular Epithelioid Cell Neoplasm

GO Terms for Perivascular Epithelioid Cell Neoplasm

Sources for Perivascular Epithelioid Cell Neoplasm

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Mar-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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