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Kidney: Papillary renal cell carcinoma

Written1998-05Jérome Couturier
Department of Pathology, Institut Curie, Paris, France

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ICD-Morpho 8260/3 Papillary adenocarcinoma, NOS
Atlas_Id 5003
Phylum Urinary system: Kidney::Renal cell carcinoma
WHO/OMS Classification Urinary system
Other nameschromophilic renal cell carcinoma


Note renal cell carcinomas form a heterogeneous group of tumours which can be divided into several subgroups according to their cytology, architecture, and the part of the nephron from which tumour cells are derived: five basic cell types are recognized: clear-cell, chromophilic-cell (also called papillary renal cell carcinomas (PRCC), herein described), chromophobic-cell, oncocytic-cell, and collector duct cell types a certain correlation exists between cell type and architecture of the tumour: the clear-cell type tends to show compact growth, while the chromophilic-cell type is more readily associated with tubulo-papillary architecture

Clinics and Pathology

Etiology a hereditary form, called Hereditary papillary renal cell carcinoma, of autosomal dominant transmission, has been recently recognized; it predisposes to develop multiple, bilateral renal tumours
Epidemiology papillary renal cell carcinomas (PRCC) represent about 10% of all renal cell tumours; there is a clear excess of male patients (male to female ratio: 5 to 1)
  • papillary renal tumours are composed of at least 50% of papillary structures, formed by connective tissue stalks covered by small or medium size cuboid cells with eosinophilic or basophilic granular cytoplasm
  • renal cortical adenomas are frequently associated with PRCC in the same kidney, suggesting the possibility of transformation from adenoma to carcinoma
  • Cytogenetics

    Note except a very rare subtype characterized by a translocation t(X;1)(p11;q21) or other abnormalities involving Xp11, PRCC do not show a recurrent structural chromosome rearrangement, but display non random numerical abnormalities; this contrasts with clear-cell renal cell carcinomas which are associated with various deletions of 3p PRCC are characterized by loss of Y chromosome in men, trisomy or tetrasomy 7 and 17, trisomies 16, 20, and 12, by order of frequency; these chromosome imbalances have been confirmed by genomic comparative hybridization multifocal PRCC show the same numerical anomalies, with trisomies sometimes in various combinations in tumours within the same kidney or in both kidneys the confrontation of karyotypic abnormalities with histopathological data suggest that specific chromosome imbalances may make it possible to distinguish benign papillary adenoma from malignant PRCC; the small papillary tumours without any morphological or clinical sign of malignancy are characterized by a combination of loss of Y, and excess of chromosomes 7 and 17, only, whereas tumours with aggressive growth and metastatic dissemination show more complex karyotypic changes, in addition to polysomy 7 and 17; it is suggested that tumours with tri- or tetrasomy 7 and 17 correspond to papillary adenomas, and that tumours with more complex karyotypic changes are papillary carcinomas, irrespective of their size

    Genes involved and Proteins

    Note in hereditary PRCC, linkages studies have shown that the gene of the disease could be located at 7q31.1-34, between markers D7S496 and D7S1837, an interval containing the MET proto-oncogene; missense mutations in the tyrosine kinase domain of the MET gene have been identified in the germline of affected members of PRCC families, and in a subset of sporadic PRCC


    Chromosome imbalances in papillary renal cell carcinoma and first cytogenetic data of familial cases analyzed by comparative genomic hybridization.
    Bentz M, Bergerheim US, Li C, Joos S, Werner CA, Baudis M, Gnarra J, Merino MJ, Zbar B, Linehan WM, Lichter P
    Cytogenetics and cell genetics. 1996 ; 75 (1) : 17-21.
    PMID 8995481
    Translocation (X;1) reveals metastasis 31 years after renal cell carcinoma.
    Dal Cin P, Stas M, Sciot R, De Wever I, Van Damme B, Van den Berghe H
    Cancer genetics and cytogenetics. 1998 ; 101 (1) : 58-61.
    PMID 9460502
    Cytogenetic investigation of synchronous bilateral renal tumors.
    Dal Cin P, van Poppel H, van Damme B, Baert L, Van den Berghe H
    Cancer genetics and cytogenetics. 1996 ; 89 (1) : 57-60.
    PMID 8689612
    Nonpapillary and papillary renal cell carcinoma: a cytogenetic and phenotypic study.
    Hughson MD, Johnson LD, Silva FG, Kovacs G
    Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc. 1993 ; 6 (4) : 449-456.
    PMID 8415591
    Molecular cytogenetics of renal cell tumors.
    Kovacs G
    Advances in cancer research. 1993 ; 62 : 89-124.
    PMID 8109322
    Cytogenetics of papillary renal cell tumors.
    Kovacs G, Fuzesi L, Emanual A, Kung HF
    Genes, chromosomes & cancer. 1991 ; 3 (4) : 249-255.
    PMID 1958590
    Germline and somatic mutations in the tyrosine kinase domain of the MET proto-oncogene in papillary renal carcinomas.
    Schmidt L, Duh FM, Chen F, Kishida T, Glenn G, Choyke P, Scherer SW, Zhuang Z, Lubensky I, Dean M, Allikmets R, Chidambaram A, Bergerheim UR, Feltis JT, Casadevall C, Zamarron A, Bernues M, Richard S, Lips CJ, Walther MM, Tsui LC, Geil L, Orcutt ML, Stackhouse T, Lipan J, Slife L, Brauch H, Decker J, Niehans G, Hughson MD, Moch H, Storkel S, Lerman MI, Linehan WM, Zbar B
    Nature genetics. 1997 ; 16 (1) : 68-73.
    PMID 9140397
    Histopathology and classification of renal cell tumors (adenomas, oncocytomas and carcinomas). The basic cytological and histopathological elements and their use for diagnostics.
    Thoenes W, Störkel S, Rumpelt HJ
    Pathology, research and practice. 1986 ; 181 (2) : 125-143.
    PMID 3737468


    This paper should be referenced as such :
    Couturier, J
    Kidney: Papillary renal cell carcinoma
    Atlas Genet Cytogenet Oncol Haematol. 1998;2(4):146-147.
    Free journal version : [ pdf ]   [ DOI ]
    On line version :

    Other genes implicated (Data extracted from papers in the Atlas) [ 30 ]


    Translocations implicated (Data extracted from papers in the Atlas)

     t(X;1)(p11;q21) PRCC/TFE3

    External links

    Mitelman database t(X;1)(p11;q21) [CaseList]     t(X;1)(p11;q21) [Transloc - MCList]   PRCC/TFE3 Fusion - MCList]
    COSMIC[ PRCC ]   [ TFE3 ]
    arrayMap Topo ( C64) arrayMap ((UZH-SIB Zurich)   [auto + random 100 samples .. if exist ]   [tabulated segments]
    Mitelman databasePRCC/TFE3[MCList]    PRCC (1q23.1) TFE3 (Xp11.23)   t(X;1)(p11;q23)
    Other databaseICGC Data Portal - [KIRP-US] Kidney Renal Papillary Cell Carcinoma - TCGA, US
    Other databaseRenal clear cell carcinoma ( intOGen )
    Other databaseKidney Chromophobe (TCGA)(OASIS Portal)
    Other databaseKidney Papillary Cell Carcinoma (TCGA)(OASIS Portal)
    Other databaseKidney Cancer Overview - Disease Synopsis [canSAR]
    Other databaseKidney Renal Papillary Cell Carcinoma [ Genomic Data Commons - NCI TCGA-KIRP]
    Disease databaseKidney: Papillary renal cell carcinoma
    REVIEW articlesautomatic search in PubMed
    Last year articlesautomatic search in PubMed

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