Synonyms
Clear cell tubulopapillary renal cell carcinoma
Definition
Indolent renal cell tumor made up of bland clear cells arranged in tubules and papillae with often linear nuclear alignment away from the basement membrane (Massari et al. 2018).
Clinical Features
Incidence
This neoplasm accounts for 1–4% of renal tumors. Most tumors are incidentally detected as sporadic or arising in end-stage renal disease and von Hippel-Lindau syndrome (Tickoo et al. 2006; Gobbo et al. 2008).
Age
There is no age predilection.
Sex
There is no gender predilection.
Site
There is no site predilection.
Treatment
Partial nephrectomy and less frequently, radical nephrectomy is the standard treatment.
Outcome
To date, neither recurrence nor metastasis has been reported (Massari et al. 2018).
Macroscopy
Tumors are often small (usually pT1a), grayish, well-circumscribed and encapsulated with common cystic change (Fig. 1a).
A small, well-circumscribed, and...
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References and Further Reading
Gobbo, S., Eble, J. N., Grignon, D. J., et al. (2008). Clear cell papillary renal cell carcinoma: a distinct histopathologic and molecular genetic entity. The American Journal of Surgical Pathology, 32, 1239–1245.
Martignoni, G., Brunelli, M., Segala, D., et al. (2017). Validation of 34betaE12 immunoexpression in clear cell papillary renal cell carcinoma as a sensitive biomarker. Pathology, 49, 10–18.
Massari, F., Ciccarese, C., Hes, O., et al. (2018). The Tumor Entity Denominated “clear cell-papillary renal cell carcinoma” According to the WHO 2016 new Classification, have the Clinical Characters of a Renal Cell Adenoma as does Harbor a Benign Outcome. Pathology Oncology Research, 24, 447–456.
Rohan, S. M., Xiao, Y., Liang, Y., et al. (2011). Clear-cell papillary renal cell carcinoma: molecular and immunohistochemical analysis with emphasis on the von Hippel-Lindau gene and hypoxia-inducible factor pathway-related proteins. Modern Pathology, 24, 1207–1220.
Tickoo, S. K., dePeralta-Venturina, M. N., Harik, L. R., et al. (2006). Spectrum of epithelial neoplasms in end-stage renal disease: an experience from 66 tumor-bearing kidneys with emphasis on histologic patterns distinct from those in sporadic adult renal neoplasia. The American Journal of Surgical Pathology, 30, 141–153.
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Caliò, A., Segala, D., Martignoni, G. (2020). Clear Cell Papillary Renal Cell Carcinoma. In: Raspollini, M.R., Lopez-Beltran, A. (eds) Uropathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-030-41894-6_4791
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DOI: https://doi.org/10.1007/978-3-030-41894-6_4791
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