Update on selected renal cell tumors with clear cell features. With emphasis on multilocular cystic clear cell renal cell carcinoma

Rodolfo Montironi, R. Mazzucchelli, M. Scarpelli, A. Lopez-Beltran, Liang Cheng

Research output: Contribution to journalArticle

13 Citations (Scopus)

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 reasons.

Original languageEnglish
Pages (from-to)1555-1566
Number of pages12
JournalHistology and Histopathology
Volume28
Issue number12
StatePublished - Dec 2013

Fingerprint

Renal Cell Carcinoma
Kidney
Neoplasms
Metaplasia
Smooth Muscle
Kidney Neoplasms
Carcinoma
Cytoplasm
Genetic Translocation
Cell Differentiation
Differential Diagnosis
Transcription Factors

Keywords

  • Clear cell (tubulo) papillary renal cell carcinoma/clear cell papillary renal cell carcinoma
  • Clear cell renal cell carcinoma
  • Multilocular cystic CCRCC
  • Xp11 translocation renal cell carcinoma

ASJC Scopus subject areas

  • Histology
  • Pathology and Forensic Medicine

Cite this

Update on selected renal cell tumors with clear cell features. With emphasis on multilocular cystic clear cell renal cell carcinoma. / Montironi, Rodolfo; Mazzucchelli, R.; Scarpelli, M.; Lopez-Beltran, A.; Cheng, Liang.

In: Histology and Histopathology, Vol. 28, No. 12, 12.2013, p. 1555-1566.

Research output: Contribution to journalArticle

Montironi, Rodolfo ; Mazzucchelli, R. ; Scarpelli, M. ; Lopez-Beltran, A. ; Cheng, Liang. / Update on selected renal cell tumors with clear cell features. With emphasis on multilocular cystic clear cell renal cell carcinoma. In: Histology and Histopathology. 2013 ; Vol. 28, No. 12. pp. 1555-1566.
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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 reasons.",
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AB - 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 reasons.

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