Publication: Update on selected renal cell tumors with clear cell features. With emphasis on multilocular cystic clear cell renal cell carcinoma
Authors
Montironi, R. ; Mazzucchelli, R. ; Scarpelli, M. ; Lopez-Beltran, A. ; Cheng, L.
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Publisher
F. Hernández y Juan F. Madrid. Universidad de Murcia. Departamento de Biología Celular e Histología
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DOI
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info:eu-repo/semantics/article
Description
Abstract
Clear cell renal cell carcinoma (CCRCC) is
the most common malignant tumor of renal epithelial
origin and, with the exception of some rare tumors, the
most deadly. The exception is represented by the
multilocular cystic CCRCC, whose prognosis is
excellent with survival rates of 100% when diagnosis is
made according to the WHO definition. For this reason a
proposal has been made to rename this tumor as
multilocular cystic renal cell neoplasm of low malignant
potential. Another exemption could be the clear cell
(tubulo) papillary renal cell carcinoma/clear cell
papillary renal cell carcinoma (CCPRCC), a tumor with
tubulopapillary architecture and clear cytoplasm.
Published data indicates that these are neoplasms with
indolent clinical behavior. No cases with metastasis have
been reported. Neoplasms meeting criteria for CCPRCC
will subsequently be reclassified as of “low malignant
potential” rather than carcinoma. The stroma of
CCPRCC not infrequently demonstrates smooth muscle
metaplasia. It should be remembered, however, that
smooth muscle stromal metaplasia and proliferation are
not entirely specific to this entity. Hence, it is suggested
that smooth muscle metaplasia in the kidney may be a
nonspecific common reaction to a variety of stimuli.
Xp11 translocation renal cell carcinomas are a group of
neoplasms distinguished by chromosomal translocations
with breakpoints involving the TFE3 transcription factor gene, which maps to the Xp11.2 locus. The most
distinctive histologic pattern of the Xp11 translocation
renal cell carcinoma is that of a neoplasm with both clear
cells and papillary architecture, and abundant
psammoma bodies. TFE3 immunohistochemical staining
is reported to be sensitive and specific for a diagnosis of
translocation-associated carcinoma as long as the
labeling is strong, diffuse, and nuclear. This
immunostaining is particularly useful if the differential
diagnosis includes CCRCC and CCPRCC. In
conclusion, recognition of CCRCC and differentiation
from other renal cell neoplasms with clear cytoplasm is
important not only for prognostication but also for
treatment-related reason
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Citation
Histology and Histopathology, vol. 28, nº 12 (2013)
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