Kidney: Papillary renal cell carcinoma

1998-05-01   Jérôme Couturier 

Classification

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 typesa 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)

Pathology

  • 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 3pPRCC 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 hybridizationmultifocal PRCC show the same numerical anomalies, with trisomies sometimes in various combinations in tumours within the same kidney or in both kidneysthe 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

    Bibliography

    Pubmed IDLast YearTitleAuthors
    89954811996Chromosome imbalances in papillary renal cell carcinoma and first cytogenetic data of familial cases analyzed by comparative genomic hybridization.Bentz M et al
    94605021998Translocation (X;1) reveals metastasis 31 years after renal cell carcinoma.Dal Cin P et al
    86896121996Cytogenetic investigation of synchronous bilateral renal tumors.Dal Cin P et al
    84155911993Nonpapillary and papillary renal cell carcinoma: a cytogenetic and phenotypic study.Hughson MD et al
    81093221993Molecular cytogenetics of renal cell tumors.Kovacs G et al
    19585901991Cytogenetics of papillary renal cell tumors.Kovacs G et al
    91403971997Germline and somatic mutations in the tyrosine kinase domain of the MET proto-oncogene in papillary renal carcinomas.Schmidt L et al
    37374681986Histopathology and classification of renal cell tumors (adenomas, oncocytomas and carcinomas). The basic cytological and histopathological elements and their use for diagnostics.Thoenes W et al

    Citation

    Jérôme Couturier

    Kidney: Papillary renal cell carcinoma

    Atlas Genet Cytogenet Oncol Haematol. 1998-05-01

    Online version: http://atlasgeneticsoncology.org/solid-tumor/5003/kidney-papillary-renal-cell-carcinoma