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Presentation & paraneoplastic syndromes

The majority (at least 60 %) of RCCs are found incidentally on imaging.

The classic triad (“too late triad”) of flank pain, haematuria and a palpable mass is now rarely seen.

Symptoms from RCC may arise from:

  • Local tumour growth
    • Haematuria
    • Flank pain
    • Abdominal mass
    • Lower limb oedema, from IVC obstruction
    • Non reducing, or right sided varicocele
  • Haemorrhage
    • 50 % of spontaneous renal bleeds may be associated with a tumour (AML > RCC)
  • Paraneoplastic syndromes
  • Metastatic disease
    • Weight loss
    • Fever
    • Night sweats

 

Paraneoplastic syndromes

Symptoms due to the systemic effects of a malignant tumour, but not due to metastatic disease or the tumour itself – usually due to substances secreted by the tumour.

10 – 20 % of patients with RCC have a paraneoplastic syndrome.

They are more common in metastatic disease and larger tumours.

They are a result of secretion of excess or pathologic secretion of either normal renal substances (1,25-dihydroxycholecalciferol), renin, EPO, prostaglandins), or other factors (PTH-like peptides, HCG, insulin etc).

Elevated ESR Most common
Hypercalcaemia Up to 13 % – either paraneoplastic (PTH like peptides), or bone metastases

Needs medical management – hydration, Lasix, bisphosphanates

Hypertension Renin production, encasement of renal arteries, AV fistula
Polycythaemia Increased EPO production
Stauffer syndrome 3 – 20 % – elevated ALP most common, as well as elevated bilirubin, transaminases, prothrombin time

No liver metastatic disease.

Weight loss, cachexia  
Anaemia  
Fever Cytokines
Cushing syndrome ACTH secretion
Hyperglycaemia Insulin or glucagon secretion
Galactorrhoea Prolactin secretion
Neuromyopathy  
Cerebellar ataxia  

 

Hypercalcaemia

Up to 13 % or more of RCC patients.

Paraneoplastic due to secretion of PTH like substances or can be due to effects of metastases on bone.

Symptoms and signs can be non-specific:

  • Abdominal pains
  • Nausea and vomiting
  • Depressed tendon reflexes
  • Anorexia
  • Fatigue
  • Confusion
  • Constipation
  • Renal stones
  • Arrhythmia
  • Myopathy

Treatment:

  • Invite (renal) physician input
  • IV hydration first line
  • Followed by diuresis with frusemide
  • Bisphosphonates next line standard of care
    • Zoledronic acid seems to work very well in RCC related hypercalcaemia
  • Other options include steroids or calcitonin
  • Definitive treatment may require nephrectomy +/- metastasectomy
  • Consider radiation if bone involvement is thought to be the cause