Clear Cell Papillary Renal Cell Carcinoma

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Retrieved

2021-01-23

Source

Orphanet

Trials

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Genes

PBRM1, MITF, HNF1A, VHL, ELOC, CA9, KRT7, CDC73, AMACR, VDR, SDHB, SLC2A1, EGFR, MME, KRT14, KRAS, HIF1A, GATA3, MIR1202

Drugs

(R, S)-3-(bromomethyl)-3-butanol-1-yl-diphosphate, 26 base single stranded phosphodiester DNA oligonucleotide, 4-amino-5-oxo-4 (pyridinium-1-ylmethyl) proline

(R, S)-3-(bromomethyl)-3-butanol-1-yl-diphosphate, 26 base single stranded phosphodiester DNA oligonucleotide, 4-amino-5-oxo-4 (pyridinium-1-ylmethyl) proline, Aldesleukin ( PROLEUKIN ), Autologous Tumor-Derived gp96 Heat Shock Protein-Peptide Complex ( ONCOPHAGE ), Autologous renal cell tumor vaccine, Axitinib ( INLYTA ), Bevacizumab ( Equidacent, AVASTIN, Aybintio, MVASI ), Everolimus ( AFINITOR, VOTUBIA ), Genetically modified allogeneic (human) tumour cells for the expression of IL-7, GM-CSF, CD80 and CD154, in fixed combination with a DNA-based double stem loop immunomodulator (dSLIM), Girentuximab ( RENCAREX ), HLA class I/II binding tumour associated peptides (ADF-APO-CCN-GUC-K67-MET-MMPMMPMUC-RGS), Human interleukin-2 (glycosylated tetrasaccharide, glycosylated trisaccharide and nonglycosylated) (inhalation use), Humanized anti-KSA monoclonal antibody-human interleukin-2 fusion protein, Interferon gamma 1b ( ACTIMMUNE, IMUKIN ), Iodine (131I) chimeric IgG monoclonal antibody cG250, N-{2-Chloro-4-[(6,7-dimethoxy-4-quinolyl)oxy]phenyl}-N'-(5-methyl-3-isoxazolyl) urea hydrochloride monohydrate, Naptumomab estafenatox, Pazopanib hydrochloride ( VOTRIENT ), Recombinant human interleukin-21, Recombinant modified vaccinia Ankara expressing human 5T4, Siltuximab ( SYLVANT ), Sorafenib tosylate ( NEXAVAR ), Sunitinib malate ( SUTENT ), Talactoferrinum alfa, Temsirolimus ( TORISEL )

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Clear cell papillary renal cell carcinoma is a rare, indolent subtype of clear cell renal carcinoma, arising from epithelial cells in the renal cortex. It most frequently manifests with a well-circumscribed, well-encapsulated, unicentric, unilateral, small tumor that typically does not metastasize. Clinically it can present with flank or abdominal pain or hematuria, although most patients are usually asymptomatic at the time of diagnosis. Bilateral and/or multifocal presentation should raise the suspicion of von Hippel-Lindau syndrome.