1. By Dr. Abeer Elsayed Aly Lecturer of medical oncology SECI 18/3/2013
Renal cell carcinoma By
Dr. Abeer Elsayed Aly
Lecturer of medical oncology
SECI
18/3/2013

5. von Hippel-Lindau syndrome
Autosomal dominant
Multiple cancers
RCC in nearly 40% of patients
del (3p) or t (3;6) or t (3;8) => VHL gene (3p26-p25) mutated => accumulation of hypoxia inducible factors (HIFs) that stimulate angiogenesis through VEGF and VEGFR

7. Hereditary papillary renal carcinomaAD
bilateral, multifocal papillary renal carcinoma
Germline missense mutations in the tyrosine kinase domain of the MET gene (7q31) => constitutive activation
TFE3 mutation
t(X;1)(p11;q21)

8. MET
β-subunit of c-Met product is the cell-surface receptor for hepatocyte growth factor
amplified during the transition between primary tumors and metastasis
metastatic potential relies on the properties of its multifunctional docking site
PRC commonly show trisomy of ch.7

9. Familial renal oncocytoma
usually benign tumors
Ultrastructural characterization exhibits dense packing of the cells with mitochondria that show morphologic differences from those in normal cells [larger, abnormally shaped]
Mutation in mtDNA within the CyC oxidase subunit I gene

10. BIRT-HOGG-DUBE SYNDROME
BHD gene 17p11.2 [TS]
Adult onset
male-to-male transmission

27. Treatment of early stages

28. Nephrectomy

35. What about adjuvant therapy?

37. Treatment of metastatic disease

39. Immunotherapy
IL2
interferon alpha

44. Alpha-methylacyl-CoA racemase
significant increase of AMACR mRNA levels in papillary renal cell carcinomas only
Also molecular marker for prostate cancer
B7-H1
expression may indicate worse survival, possibly through impaired host antitumor immunity
Microsatellite instabilities

47. Target Therapy What is my the target?

59. Temsirolimus

64. Bevaciziumab

76. Pazopanib

82. Vaccine

84. Reduced inteinsty myeloablative bone marrow transplant

86. Summary

92. Thank You