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Kidney Mixed Epithelial and Stromal Tumor Family

MedGen UID:
1684226
•Concept ID:
C4764006
•
Neoplastic Process
Synonym: MEST Family

Definition

A family of tumors ranging from predominantly cystic tumors (adult cystic nephromas) to tumors that are variably solid (mixed epithelial and stromal tumors) and contain biphasic epithelial and stromal components with spindle stroma, glands, and cysts. Most of these tumors are benign. (WHO 2016). [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVKidney Mixed Epithelial and Stromal Tumor Family

Recent clinical studies

Etiology

A two-generation hyperparathyroidism-jaw tumor (HPT-JT) syndrome family: clinical presentations, pathological characteristics and genetic analysis: a case report.
Yang D, Zheng J, Tang F, He Q, Huang H, Zhou P
Diagn Pathol 2022 Sep 24;17(1):71. doi: 10.1186/s13000-022-01248-x. PMID: 36153594Free PMC Article
Mixed Epithelial and Stromal Tumor Family of Kidney (Adult Cystic Nephroma, Mixed Epithelial and Stromal Tumor): Retrospective Clinicopathological Evaluation.
Demir H, Sahin Z, Ozman O, Demirbilek M, Ozden SB, Gurses I, Durak H, Uygun N, Onal B
Turk Patoloji Derg 2022;38(3):251-260. doi: 10.5146/tjpath.2022.01575. PMID: 35642344Free PMC Article
CDC73 Germline Mutation in a Family With Mixed Epithelial and Stromal Tumors.
Vocke CD, Ricketts CJ, Ball MW, Schmidt LS, Metwalli AR, Middelton LA, Killian JK, Khan J, Meltzer PS, Simonds WF, Merino MJ, Linehan WM
Urology 2019 Feb;124:91-97. Epub 2018 Nov 16 doi: 10.1016/j.urology.2018.11.013. PMID: 30452964Free PMC Article
The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia.
Srigley JR, Delahunt B, Eble JN, Egevad L, Epstein JI, Grignon D, Hes O, Moch H, Montironi R, Tickoo SK, Zhou M, Argani P; ISUP Renal Tumor Panel
Am J Surg Pathol 2013 Oct;37(10):1469-89. doi: 10.1097/PAS.0b013e318299f2d1. PMID: 24025519
2009 update on the classification of renal epithelial tumors in adults.
Lopez-Beltran A, Carrasco JC, Cheng L, Scarpelli M, Kirkali Z, Montironi R
Int J Urol 2009 May;16(5):432-43. Epub 2009 Apr 20 doi: 10.1111/j.1442-2042.2009.02302.x. PMID: 19453547

Diagnosis

Mixed epithelial and stromal tumour of the kidney masquerading as hydatid cyst: a rare case report from the developing world.
Mahar NA, Hussain M, Shehzad Shah SA, Qureshi HH, Kalwar SR, Sultan G
J Pak Med Assoc 2023 May;73(5):1117-1119. doi: 10.47391/JPMA.6876. PMID: 37218248
Mixed Epithelial and Stromal Tumor Family of Kidney (Adult Cystic Nephroma, Mixed Epithelial and Stromal Tumor): Retrospective Clinicopathological Evaluation.
Demir H, Sahin Z, Ozman O, Demirbilek M, Ozden SB, Gurses I, Durak H, Uygun N, Onal B
Turk Patoloji Derg 2022;38(3):251-260. doi: 10.5146/tjpath.2022.01575. PMID: 35642344Free PMC Article
CDC73 Germline Mutation in a Family With Mixed Epithelial and Stromal Tumors.
Vocke CD, Ricketts CJ, Ball MW, Schmidt LS, Metwalli AR, Middelton LA, Killian JK, Khan J, Meltzer PS, Simonds WF, Merino MJ, Linehan WM
Urology 2019 Feb;124:91-97. Epub 2018 Nov 16 doi: 10.1016/j.urology.2018.11.013. PMID: 30452964Free PMC Article
2009 update on the classification of renal epithelial tumors in adults.
Lopez-Beltran A, Carrasco JC, Cheng L, Scarpelli M, Kirkali Z, Montironi R
Int J Urol 2009 May;16(5):432-43. Epub 2009 Apr 20 doi: 10.1111/j.1442-2042.2009.02302.x. PMID: 19453547
2004 WHO classification of the renal tumors of the adults.
Lopez-Beltran A, Scarpelli M, Montironi R, Kirkali Z
Eur Urol 2006 May;49(5):798-805. Epub 2006 Jan 17 doi: 10.1016/j.eururo.2005.11.035. PMID: 16442207

Prognosis

2009 update on the classification of renal epithelial tumors in adults.
Lopez-Beltran A, Carrasco JC, Cheng L, Scarpelli M, Kirkali Z, Montironi R
Int J Urol 2009 May;16(5):432-43. Epub 2009 Apr 20 doi: 10.1111/j.1442-2042.2009.02302.x. PMID: 19453547

Clinical prediction guides

Mixed Epithelial and Stromal Tumor Family of Kidney (Adult Cystic Nephroma, Mixed Epithelial and Stromal Tumor): Retrospective Clinicopathological Evaluation.
Demir H, Sahin Z, Ozman O, Demirbilek M, Ozden SB, Gurses I, Durak H, Uygun N, Onal B
Turk Patoloji Derg 2022;38(3):251-260. doi: 10.5146/tjpath.2022.01575. PMID: 35642344Free PMC Article

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