Makindo Medical Notes

Related Subjects: |Urothelial tumour s |Haematuria |Acute Urinary Retention |Anuria and Oliguria |Bladder cancer |Renal cell carcinoma |Benign Prostatic Hyperplasia |IgA nephropathy |Prostate Cancer |Henoch-Schonlein purpura |Glomerulonephritis

About

• Renal cell carcinoma (RCC) is the most common renal tumour in adults.
• Primarily affects individuals aged 50+ and is more common in males.

Aetiology

• Genetic Factors:
  • Von Hippel-Lindau (VHL) syndrome: A hereditary condition associated with mutations in the VHL tumour suppressor gene.
  • Hereditary Papillary Renal Carcinoma (HPRC): An autosomal dominant disorder predisposing individuals to RCC.
  • Other genetic syndromes include Birt-Hogg-Dubé syndrome and hereditary leiomyomatosis.
• Lifestyle Factors:
  • Smoking: A significant risk factor for RCC.
  • Obesity: Increases the risk of developing RCC.
• Medical Conditions:
  • Hypertension: Associated with an increased risk of RCC.
  • Chronic kidney disease and long-term dialysis are also predisposing factors.
• Occupational Exposures:
  • Exposure to chemicals like asbestos and cadmium increases RCC risk.

Histology

• Originates in the renal tubular epithelium; 10% of cases are bilateral.
• Encompasses over 10 histological and molecular subtypes.
• Clear cell RCC (ccRCC): The most common subtype, accounting for the majority of cancer-related deaths.

Risk Factors

• Male gender (higher prevalence compared to females).
• Obesity, hypertension, smoking, chronic kidney disease.
• Haemodialysis, kidney transplantation, acquired cystic kidney disease.
• Previous RCC diagnosis and possibly diabetes mellitus.

Clinical Features

• Symptoms: Flank pain, haematuria, abdominal mass, weight loss, fever, and polycythaemia.
• Paraneoplastic effects: Ectopic production of hormones like erythropoietin (EPO), ACTH, PTHrp, prolactin, and renin.
• Left-sided varicocele due to compression of the left testicular vein.
• Metastases commonly affect the lungs, liver, bones, and brain.
• May invade the renal vein, extend into the inferior vena cava, and spread haematogenously.

Investigations

• Urinalysis: Detects blood and malignant cells.
• FBC: May reveal anaemia or polycythaemia.
• Imaging:
  • Ultrasound (USS): Identifies renal masses and venous involvement.
  • CT Scan: Critical for staging and detailed imaging.
  • MRI: Useful for evaluating tumour spread, particularly in complex cases.
• CXR: To detect "cannonball" metastases.
• Renal Biopsy: Confirms diagnosis and subtype.

Renal cell carcinoma (RCC) originates from the epithelium of the renal tubules, with approximately 10% of cases being bilateral. RCC is a highly heterogeneous disease with more than 10 distinct histological and molecular subtypes. The most common subtype is clear cell RCC (ccRCC), which accounts for 75–80% of cases and is associated with mutations in the Von Hippel-Lindau (VHL) gene.

Management

• General Measures: Smoking cessation and weight management.
• Surgery:
  • Partial nephrectomy: Preserves renal function in localized disease.
  • Radical nephrectomy: Involves removal of the kidney, adrenal gland, and nearby lymph nodes.
• Local Treatments:
  • Radiofrequency ablation (RFA): Uses heat to destroy cancer cells.
  • Cryoablation: Destroys cancer cells with extreme cold.
• Radiation Therapy: Primarily used for palliation in metastatic disease.
• Immunotherapy: Checkpoint inhibitors like nivolumab and pembrolizumab boost immune response.
• Targeted Therapy: Drugs like tyrosine kinase inhibitors (e.g., sunitinib) target cancer-specific pathways.
• Prognosis: 5-year survival rates:
  • Localized disease: 60%.
  • Metastatic disease: ~5%.

References

• Renal Cell Carcinoma - ResearchGate
• Kidney Cancer - American Cancer Society