Mixed epithelial and stromal tumor family

2021-07-09   Paola Dal Cin 

1.Brigham and Women's Hospital , Harvard Medical School, Boston , MA (USA)

Classification

Definition

Adult cystic nephroma (ACN) and mixed epithelial and stromal tumor (MEST) are rare benign renal neoplasms that have overlapping clinical and morphologic features, including predominance in middle-aged women  Preliminary results showed that MEST/CN have  distinguishing morphologic, immunohistochemical and genetic features, with  lack of DICER1 mutation 1,2, seen in pediatric cystic nephroma, 

Summary

Mixed epithelial and stromal tumour family Genetic marker(s)
Adult cystic nephroma (ACN)DICER1 mutation status does not absolutely distinguish adult and pediatric cystic nephroma in all cases, though in the vast majority of cases it does.
Mixed epitheilial and stromal tumour family (MEST)Mixed epithelial and stromal tumor (MEST) family” to encompass both MEST and ACN, and lack of DICER1 mutation 1,2,3, seen in pediatric cystic nephroma .

Bibliography

Reference NumberPubmed IDLast YearTitleAuthors
1265099052017Mixed Epithelial and Stromal Tumor of the Kidney: Mutation Analysis of the DICER 1 Gene in 29 Cases.Vanecek T et al
2281779622017Pediatric Cystic Nephroma Is Morphologically, Immunohistochemically, and Genetically Distinct From Adult Cystic Nephroma.Li Y et al
3327570722021A MEST up classification? Review of the re-classification of mixed epithelial and stromal tumor and adult cystic nephroma for the abdominal radiologist.Magnelli LL et al

External Links

Citation

Paola Dal Cin

Mixed epithelial and stromal tumor family

Atlas Genet Cytogenet Oncol Haematol. 2021-07-09

Online version: http://atlasgeneticsoncology.org/solid-tumor/208923/mixed-epithelial-and-stromal-tumor-family