Clear Cell Papillary Renal Cell Carcinoma
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).
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).
There is no age predilection.
There is no gender predilection.
There is no site predilection.
Partial nephrectomy and less frequently, radical nephrectomy is the standard treatment.
To date, neither recurrence nor metastasis has been reported (Massari et al. 2018).
Tumors are often small (usually pT1a), grayish, well-circumscribed and encapsulated with common cystic change (Fig. 1a).
Tubular, papillary, branched glandular, acinar, and cystic patterns are seen in varying proportions. Neoplastic clear cells are cuboidal with round nuclei and inconspicuous nucleoli (ISUP/WHO grade 1 and 2) arranged in a linear fashion away from the basement membrane. Fibrous and/or smooth muscle stroma in varying amounts may be observed (Fig. 1b).
These tumors lack the classic genetic events of clear cell renal cell carcinoma (3p/VHL alteration) and papillary renal cell carcinoma (7 and 17 trisomies) (Rohan et al. 2011).
- 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.CrossRefGoogle Scholar
- 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.CrossRefGoogle Scholar