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Bladder Malignant PEComa

MedGen UID:
1696136
•Concept ID:
C5204618
•
Neoplastic Process
Synonym: Bladder Malignant Perivascular Epithelioid Cell Tumor

Definition

A malignant perivascular epithelioid cell tumor arising from the bladder. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBladder Malignant PEComa

Recent clinical studies

Etiology

Histopathological spectrum of adult renal tumours: A single center experience and review of literature.
Sheenu VS, Paul RS, Pushpa M, Ami EM, Rashmi R, Iona ML, Elizabeth M
Indian J Pathol Microbiol 2023 Apr-Jun;66(2):307-313. doi: 10.4103/ijpm.ijpm_53_21. PMID: 37077073
PEComas of the kidney and of the genitourinary tract.
Martignoni G, Pea M, Zampini C, Brunelli M, Segala D, Zamboni G, Bonetti F
Semin Diagn Pathol 2015 Mar;32(2):140-59. Epub 2015 Feb 24 doi: 10.1053/j.semdp.2015.02.006. PMID: 25804448
Dichotomy of Genetic Abnormalities in PEComas With Therapeutic Implications.
Agaram NP, Sung YS, Zhang L, Chen CL, Chen HW, Singer S, Dickson MA, Berger MF, Antonescu CR
Am J Surg Pathol 2015 Jun;39(6):813-25. doi: 10.1097/PAS.0000000000000389. PMID: 25651471Free PMC Article
PEComa of the urinary bladder.
Shringarpure SS, Thachil JV, Maya M
Saudi J Kidney Dis Transpl 2012 Sep;23(5):1032-4. doi: 10.4103/1319-2442.100943. PMID: 22982918
Bladder perivascular epithelioid cell tumor: a novel rare neoplasm.
Kyrou D, Staios D, Papatsoris AG
Urol Int 2012;88(4):480-2. Epub 2011 Nov 15 doi: 10.1159/000332199. PMID: 22094379

Diagnosis

PEComa-like Neoplasms Characterized by ASPSCR1-TFE3 Fusion: Another Face of TFE3-related Mesenchymal Neoplasia.
Argani P, Wobker SE, Gross JM, Matoso A, Fletcher CDM, Antonescu CR
Am J Surg Pathol 2022 Aug 1;46(8):1153-1159. Epub 2022 Apr 5 doi: 10.1097/PAS.0000000000001894. PMID: 35848761Free PMC Article
Perivascular Epithelioid Cell Tumor of Urinary Bladder.
Wu YL, Lang L, Ma Q, Wu F, Zhang Y, Chen W, Yan XC, Duan GJ
Am J Clin Pathol 2021 Jun 17;156(1):56-71. doi: 10.1093/ajcp/aqaa204. PMID: 33336255
Giant multilocular prostatic cystadenoma.
Nakamura Y, Shida D, Shibayama T, Yoshida A, Matsui Y, Shinoda Y, Iwata S, Kanemitsu Y
World J Surg Oncol 2019 Feb 26;17(1):42. doi: 10.1186/s12957-019-1579-7. PMID: 30808350Free PMC Article
Bladder perivascular epithelioid cell tumor (PEComa): a case report and literature review.
Sarti A, Rubilotta E, Balzarro M, Cerruto MA, Brunelli M, Artibani W
Urologia 2015 Apr-Jun;82(2):120-3. Epub 2014 Jan 28 doi: 10.5301/urologia.5000045. PMID: 24557821
Soft tissue tumors of the urinary bladder, Part I: myofibroblastic proliferations, benign neoplasms, and tumors of uncertain malignant potential.
Lott S, Lopez-Beltran A, Maclennan GT, Montironi R, Cheng L
Hum Pathol 2007 Jun;38(6):807-23. doi: 10.1016/j.humpath.2007.03.017. PMID: 17509394

Therapy

Bone metastases from bladder perivascular epithelioid cell tumor - an unusual localization of a rare tumor: a case report.
Palleschi G, Pastore AL, Evangelista S, Silvestri L, Rossi L, Di Cristofano C, Porta N, Petrozza V, Tomao S, Carbone A
J Med Case Rep 2014 Jun 25;8:227. doi: 10.1186/1752-1947-8-227. PMID: 24965209Free PMC Article
New embolization microcoil consisting of firm and flexible segments: preliminary clinical experience.
Irie T
Cardiovasc Intervent Radiol 2006 Nov-Dec;29(6):986-90. doi: 10.1007/s00270-005-0364-x. PMID: 16967218
Primary PEComa of the bladder treated with primary excision and adjuvant interferon-alpha immunotherapy: a case report.
Parfitt JR, Bella AJ, Wehrli BM, Izawa JI
BMC Urol 2006 Aug 22;6:20. doi: 10.1186/1471-2490-6-20. PMID: 16925812Free PMC Article

Prognosis

Application of Circular RNA Circ_0071662 in the Diagnosis and Prognosis of Hepatocellular Carcinoma and Its Response to Radiotherapy.
Wang X, Zhang J, Luo F, Shen Y
Dig Dis 2023;41(3):431-438. Epub 2022 Oct 24 doi: 10.1159/000527696. PMID: 36279855
Perivascular Epithelioid Cell Tumor of Urinary Bladder.
Wu YL, Lang L, Ma Q, Wu F, Zhang Y, Chen W, Yan XC, Duan GJ
Am J Clin Pathol 2021 Jun 17;156(1):56-71. doi: 10.1093/ajcp/aqaa204. PMID: 33336255
Perivascular Epithelioid Cell Tumor of the Urinary Bladder: A Systematic Review.
Hamza A, Guo CC
Int J Surg Pathol 2020 Jun;28(4):393-400. Epub 2019 Dec 22 doi: 10.1177/1066896919895810. PMID: 31865807
Bladder Perivascular Epithelioid Cell Tumor: Dynamic CT and MRI Presentation of 2 Cases With 2-year Follow-up and Review of the Literature.
Xuesong D, Hong G, Weiguo Z
Clin Genitourin Cancer 2019 Oct;17(5):e916-e922. Epub 2019 Jul 2 doi: 10.1016/j.clgc.2019.06.016. PMID: 31327725
Malignant perivascular epithelioid cell neoplasm (PEComa) of the urinary bladder with TFE3 gene rearrangement: clinicopathologic, immunohistochemical, and molecular features.
Williamson SR, Bunde PJ, Montironi R, Lopez-Beltran A, Zhang S, Wang M, Maclennan GT, Cheng L
Am J Surg Pathol 2013 Oct;37(10):1619-26. doi: 10.1097/PAS.0b013e318293729d. PMID: 23797724

Clinical prediction guides

Transurethral en bloc resection of a bladder perivascular epithelioid cell tumor (PEComa): a case report.
Zeng SP, Sun YF, Ye JB, Zeng K, Li XB
BMC Urol 2023 Mar 2;23(1):28. doi: 10.1186/s12894-023-01198-6. PMID: 36864398Free PMC Article
Application of Circular RNA Circ_0071662 in the Diagnosis and Prognosis of Hepatocellular Carcinoma and Its Response to Radiotherapy.
Wang X, Zhang J, Luo F, Shen Y
Dig Dis 2023;41(3):431-438. Epub 2022 Oct 24 doi: 10.1159/000527696. PMID: 36279855
Incidental Pelvic and Para-aortic Lymph Node Lymphangioleiomyomatosis Detected During Surgical Staging of Pelvic Cancer in Women Without Symptomatic Pulmonary Lymphangioleiomyomatosis or Tuberous Sclerosis Complex.
Rabban JT, Firetag B, Sangoi AR, Post MD, Zaloudek CJ
Am J Surg Pathol 2015 Aug;39(8):1015-25. doi: 10.1097/PAS.0000000000000416. PMID: 25786086
Characteristics and significant predictors of detecting underlying diseases in adults with asymptomatic microscopic hematuria: a large case series of a Korean population.
Kang M, Lee S, Jeong SJ, Hong SK, Byun SS, Lee SE, Jeong CW
Int J Urol 2015 Apr;22(4):389-93. Epub 2015 Jan 12 doi: 10.1111/iju.12697. PMID: 25581719
Comparative genomic hybridization study of perivascular epithelioid cell tumor: molecular genetic evidence of perivascular epithelioid cell tumor as a distinctive neoplasm.
Pan CC, Jong YJ, Chai CY, Huang SH, Chen YJ
Hum Pathol 2006 May;37(5):606-12. doi: 10.1016/j.humpath.2006.01.008. PMID: 16647959

Recent systematic reviews

Perivascular Epithelioid Cell Tumor of the Urinary Bladder: A Systematic Review.
Hamza A, Guo CC
Int J Surg Pathol 2020 Jun;28(4):393-400. Epub 2019 Dec 22 doi: 10.1177/1066896919895810. PMID: 31865807

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