TY - JOUR
T1 - Eosinophilic Vacuolated Tumor of the Kidney
T2 - A Review of Evolving Concepts in This Novel Subtype with Additional Insights from a Case with MTOR Mutation and Concomitant Chromosome 1 Loss
AU - Kapur, Payal
AU - Gao, Ming
AU - Zhong, Hua
AU - Rakheja, DInesh
AU - Cai, Qi
AU - Pedrosa, Ivan
AU - Margulis, Vitaly
AU - Xu, Lin
AU - Kinch, Lisa
AU - Brugarolas, James
N1 - Publisher Copyright:
© 2021 Lippincott Williams and Wilkins. All rights reserved.
PY - 2021/7
Y1 - 2021/7
N2 - Recent advances in molecular genetics have expanded our knowledge of renal tumors and enabled a better classification. These studies have revealed that renal tumors with predominantly "eosinophilic/oncocytic" cytoplasm include several novel biological subtypes beyond the traditionally well-recognized renal oncocytoma and an eosinophilic variant of chromophobe renal cell carcinoma. Herein, we present a comprehensive review of the eosinophilic vacuolated tumor (EVT) building upon a case report including radiology, histopathology, electron microscopy, and next-generation sequencing. EVTs are characterized by mTORC1 activation. We speculate that loss of chromosome 1 in EVT with MTOR mutation may be driven in part by an advantage conferred by loss of the remaining MTOR wild-type allele. mTORC1 is best known for its role in promoting protein translation and it is interesting that dilated cisterns of rough endoplasmic reticulum (ER) likely account for the cytoplasmic vacuoles seen by light microscopy. We present an integrated view of EVT as well as cues that can assist in the differential diagnosis.
AB - Recent advances in molecular genetics have expanded our knowledge of renal tumors and enabled a better classification. These studies have revealed that renal tumors with predominantly "eosinophilic/oncocytic" cytoplasm include several novel biological subtypes beyond the traditionally well-recognized renal oncocytoma and an eosinophilic variant of chromophobe renal cell carcinoma. Herein, we present a comprehensive review of the eosinophilic vacuolated tumor (EVT) building upon a case report including radiology, histopathology, electron microscopy, and next-generation sequencing. EVTs are characterized by mTORC1 activation. We speculate that loss of chromosome 1 in EVT with MTOR mutation may be driven in part by an advantage conferred by loss of the remaining MTOR wild-type allele. mTORC1 is best known for its role in promoting protein translation and it is interesting that dilated cisterns of rough endoplasmic reticulum (ER) likely account for the cytoplasmic vacuoles seen by light microscopy. We present an integrated view of EVT as well as cues that can assist in the differential diagnosis.
KW - MTOR
KW - RCC with cytoplasmic eosinophilia
KW - TSC-associated RCC
KW - eosinophilic tumor
KW - eosinophilic vacuolated tumor
KW - eosinophilic variant of chromophobe renal cell carcinoma
KW - high-grade oncocytic tumor
KW - hybrid oncocytic/chromophobe RCCs
KW - unclassified oncocytic tumor
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U2 - 10.1097/PAP.0000000000000299
DO - 10.1097/PAP.0000000000000299
M3 - Review article
C2 - 34009776
AN - SCOPUS:85108303410
SN - 1072-4109
VL - 28
SP - 251
EP - 257
JO - Advances in anatomic pathology
JF - Advances in anatomic pathology
IS - 4
ER -