Eosinophilic vacuolated tumour
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Eosinophilic vacuolated tumour | |
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Diagnosis in short | |
Micrograph of a high-grade oncocytic tumour of the kidney. H&E stain. | |
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Synonyms | high-grade oncocytic tumour |
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LM |
abundant oncocytic cytoplasm with prominent intracytoplasmic vacuoles, round nuclei, prominent nucleoli (ISUP nucleolar grade 3), no perinuclear halos |
LM DDx | renal oncocytoma, renal hybrid oncocytic/chromophobe tumour, chromophobe renal cell carcinoma, other renal tumours with eosinophilic cytoplasm |
IHC | PAX8 +ve, CD117 +ve, CD10 +ve, SDHB +ve, TFE3 -ve |
Molecular | TSC1 or TSC2 mutation |
Site | kidney - see kidney tumours |
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Syndromes | tuberous sclerosis |
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Prevalence | very rare? (evolving entity) |
Prognosis | unknown (evolving entity) |
Clin. DDx | other renal tumours |
Treatment | excision |
Eosinophilic vacuolated tumour, abbreviated EVT, is a kidney tumour type currently not recognized by the WHO classification as per the 2016 Blue Book.
It was previously known as high-grade oncocytic tumour, abbreviated HOT.[1]
General
- Previously lumped into renal hybrid oncocytic/chromophobe tumour.
- Evolving entity.
- May be seen within the context of tuberous sclerosis.[2]
Microscopic
Features:[3]
- Abundant oncocytic cytoplasm with prominent intracytoplasmic vacuoles.
- Round nuclei
- No "raisinoid" nuclei typical of chromophobe RCC.
- Prominent nucleoli (ISUP nucleolar grade 3).
- No perinuclear halos.
DDx:
- Renal oncocytoma.
- Renal hybrid oncocytic/chromophobe tumour.
- Chromophobe renal cell carcinoma.
- Other renal tumours with eosinophilic cytoplasm.
Images
IHC
Features:[3]
- PAX8 +ve.
- AE1/AE3 +ve.
- CK18 +ve.
- SDHB +ve.
- CD10 +ve (12 of 13 cases).
- HMB45 -ve.
- Melan A -ve.
- TFE3 -ve.
- CD117 +ve (9 of 14 cases).
Others:
- CD56 +ve.[citation needed]
- Typically negative in renal oncocytoma.
Molecular
- TSC1 or TSC2 mutation - see renal neoplasms with TSC/mTOR pathway mutations.
See also
References
- ↑ Farcaş M, Gatalica Z, Trpkov K, Swensen J, Zhou M, Alaghehbandan R, Williamson SR, Magi-Galluzzi C, Gill AJ, Tretiakova M, Lopez JI, Montiel DP, Sperga M, Comperat E, Brimo F, Yilmaz A, Siadat F, Sangoi A, Gao Y, Ptákova N, Kuthi L, Pivovarcikova K, Rogala J, Agaimy A, Hartmann A, Fraune C, Rychly B, Hurnik P, Durcansky D, Bonert M, Gakis G, Michal M, Hora M, Hes O (September 2021). "Eosinophilic vacuolated tumor (EVT) of kidney demonstrates sporadic TSC/MTOR mutations: next-generation sequencing multi-institutional study of 19 cases". Mod Pathol. doi:10.1038/s41379-021-00923-6. PMID 34521993.
- ↑ Trpkov, K.; Bonert, M.; Gao, Y.; Kapoor, A.; He, H.; Yilmaz, A.; Gill, AJ.; Williamson, SR. et al. (Apr 2019). "High-grade oncocytic tumour (HOT) of kidney in a patient with tuberous sclerosis complex.". Histopathology. doi:10.1111/his.13876. PMID 31002177.
- ↑ 3.0 3.1 He, H.; Trpkov, K.; Martinek, P.; Isikci, OT.; Maggi-Galuzzi, C.; Alaghehbandan, R.; Gill, AJ.; Tretiakova, M. et al. (Dec 2018). ""High-grade oncocytic renal tumor": morphologic, immunohistochemical, and molecular genetic study of 14 cases.". Virchows Arch 473 (6): 725-738. doi:10.1007/s00428-018-2456-4. PMID 30232607.