Clear cell papillary renal cell carcinoma: A review

Naoto Kuroda, Chisato Ohe, Fumi Kawakami, Shuji Mikami, Mitsuko Furuya, Keiko Matsuura, Masatsugu Moriyama, Yoji Nagashima, Ming Zhou, Fredrik Petersson, José I. López, Ondrej Hes, Michal Michal, Mahul B. Amin

Research output: Contribution to journalReview articlepeer-review

31 Citations (Scopus)


The disease concept of clear cell (tubulo) papillary renal cell carcinoma (CCP-RCC) as a distinct subtype of renal cell carcinoma has been recently established. First described in the setting of end stage renal disease, this tumor type is more frequently recognized and encountered in a sporadic setting. In this article, we provide an overview of the recent understanding of this tumor. Macroscopically, tumors are well circumscribed with well-developed tumor capsule. Histologically, the tumor cells are cuboidal to low columnar cell with clear cytoplasm and papillary and tubulo-papillary configuration. Immunohistochemically, tumor cells generally show diffuse expression for cytokeratin 7, CA9 (cup-shaped pattern), HIF-1, GLUT-1 and high molecular weight cytokeratin, but negative for AMACR, RCC Ma and TFE3. CD10 is negative or focally positive in most tumors. Genetically, this tumor has no characteristics of clear cell RCC or papillary RCC. Prognostically, patients with CCP-RCC behave in an indolent fashion in all previously reported cases. In conclusion, although this tumor has been integrated into recent International Society of Urologic Pathology Classification of renal neoplasia, both aspects of disease concept and clinical behavior are yet to be fully elucidated. Further publications of large cohorts of patients will truly help understand the biologic potential and the molecular underpinnings of this tumor type.

Original languageEnglish
Pages (from-to)7312-7318
Number of pages7
JournalInternational Journal of Clinical and Experimental Pathology
Issue number11
Publication statusPublished - 2014
Externally publishedYes


  • Clear cell (tubulo) papillary renal cell carcinoma
  • Pathology

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology


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