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Renal cell carcinoma: Difference between revisions
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<section begin="radiology" />'''Renal cell carcinoma''' (RCC) is the most common renal cancer in adults, accounting for 80+%. It's a malignant epithelial tumor that arises from the kidney tubules. It's usually discovered incidentally. When symptomatic, it usually presents with a classic triad of haematuria (with normal and not dysmorphic RBCs), costovertebral pain and/or a palpable mass in the flank. | <section begin="pathology" /><section begin="radiology" />'''Renal cell carcinoma''' (RCC) is the most common histological type of '''renal cancer''' in adults, accounting for 80+%. It's a malignant epithelial tumor that arises from the kidney tubules. It's usually discovered incidentally. When symptomatic, it usually presents with a classic triad of haematuria (with normal and not dysmorphic RBCs), costovertebral pain and/or a palpable mass in the flank. | ||
It's a relatively rare malignancy, with an incidence of 15/100 000 new cases every year. It's mostly a disease of elderly men.<section end="radiology" /> | It's a relatively rare malignancy, with an incidence of 15/100 000 new cases every year. It's mostly a disease of elderly men. Most are diagnosed with localised disease. Up to 70% of cases are incidentally diagnosed on imaging.<section end="radiology" /> | ||
== | Malignancy of the renal pelvis is usually urothelial carcinoma and is therefore more similar to [[bladder cancer]]. It is not considered a renal cancer. | ||
== Risk factors == | |||
It's usually idiopathic, bue some risk factors are known: | |||
* [[Smoking]] | * [[Smoking]] | ||
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== Pathology == | == Pathology == | ||
Renal cell carcinomas are adenocarcinomas which arise from the tubular epithelium. The most common types are clear cell carcinoma (80%+ of cases) and papillary carcinoma (10% of cases). Other types include: | |||
* Chromophobe RCC – 5% of cases | * Chromophobe RCC – 5% of cases | ||
* Cystic RCC – 2.5% of cases | * Cystic RCC – 2.5% of cases | ||
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Collecting duct RCC is a very rare subtype. It’s very aggressive and has a particularly bad prognosis. | Collecting duct RCC is a very rare subtype. It’s very aggressive and has a particularly bad prognosis. | ||
=== Grading === | |||
The Furhman system is used as grading system for kidney tumors. It’s based mostly on how prominent the nucleoli are – the more prominent, the higher the grade. A grade IV RCC has ''sarcomatoid'' or ''rhabdoid'' appearance. | |||
=== Metastasis === | |||
As RCC commonly grows to the renal vein and IVC is metastasis to the lungs and bone most frequent. The tumor grows as a solid column inside the renal vein, sometimes extending as far as the IVC or the heart. As for lymph nodes it first spreads to retroperitoneal lymph nodes. | |||
RCC can also cause a left-sided [[varicocele]], a pathological dilation of the venous plexus of the scrotum. This tumor commonly spreads locally to the renal vein, where it can block the point the left testicular vein drains into the renal vein. This blockage causes pressure to build up in the venous plexus of the scrotum which may be felt as a soft lump. | |||
=== Paraneoplastic syndromes === | |||
RCC can cause various [[Paraneoplastic syndrome|paraneoplastic syndromes]]: | |||
* [[Hypertension]] – as it can produce [[renin]] | |||
* [[Hypercalcaemia]] – as it can produce [[parathyroid hormone-related protein]] (PTHrP) | |||
* [[Polycythaemia]] – as it can produce [[EPO]] | |||
* Secondary hypercortisolism ([[Cushing syndrome]]) – as it can produce ACTH | |||
* Anaemia | |||
== Clinical features == | == Clinical features == | ||
Renal cell carcinoma may be asymptomatic in the initial stages, causing it to be | Renal cell carcinoma may be asymptomatic in the initial stages, causing it to mostly be found incidentally. Up to 25% of cases already have distant metastases at diagnosis. | ||
When symptomatic, renal cell carcinoma has a typical triad of symptoms: | |||
* Haematuria (with normal and not dysmorphic RBCs) | * Haematuria (with normal and not dysmorphic RBCs) | ||
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..., however it's rare for RCC to present with the triad. It may also cause non-specific symptoms like fever and weight loss. | ..., however it's rare for RCC to present with the triad. It may also cause non-specific symptoms like fever and weight loss. | ||
== Diagnosis and evaluation == | == Diagnosis and evaluation == | ||
[[ | Non-dysmorphic [[haematuria]], is usually present. There are no specific tumour markers.<section begin="radiology" /><section end="pathology" /> | ||
=== Imaging === | === Imaging === | ||
[[CT]] with contrast is required to make the diagnosis, but [[ultrasound]] is sometimes the first imaging modality used. Ultrasound may show an expanding hyperechoic or hypoechoic process. On CT renal cell carcinoma is visible as the tumour enhances with contrast. Invasion of the renal vein is common. | [[CT]] with contrast is required to make the diagnosis, but [[ultrasound]] is sometimes the first imaging modality used. Ultrasound may show an expanding hyperechoic or hypoechoic process. On CT renal cell carcinoma is visible as the tumour enhances with contrast. Invasion of the renal vein is common. Following the diagnosis, CT of the thorax should be made to look for metastasis. | ||
<section end="radiology" /> | <section begin="pathology" /> | ||
=== Staging === | |||
The TNM staging of kidney tumors depend on the size, renal vein involvement and local invasion. 7 cm in size is the border between T1 and T2 tumours. Kidney tumors commonly infiltrate the fatty capsule and when they do they are T3. If they infiltrate beyond the renal fascia (Gerota fascia) the cancer is considered T4 and therefore inoperable.<section end="radiology" /> | |||
=== Biopsy === | |||
Biopsi is often indicated, but is not necessary if the imaging findings are very typical for malignancy. If imaging cannot rule out benign tumour, biopsy is indicated. | |||
== Management == | == Management == | ||
The main treatment is | The main treatment for non-metastatic disease is surgical removal or resection of the kidney. <section end="pathology" />Adjuvant therapy is not recommended. Some may be monitored with [[active surveillance]]. For metastatic disease, targeted therapy and immunotherapy are indicated. Complete metastasectomy may improve survival. | ||
== | === Surgical treatment === | ||
Nephron-sparing surgery refers to partial nephrectomy and is recommended over radical nephrectomy if the conditions allow for it, usually if the tumour is < 4 cm. Partial nephrectomy preserves kidney function and is especially useful when the patient only has one kidney or there are bilateral tumours. | |||
For T2-T4 tumours, radical nephrectomy is indicated. | |||
Cytoreductive nephrectomy refers to radical or partial nephrectomy in the setting of metastatic disease. The intention is to remove as many tumour cells as possible. In some cases, it may lead to regression of metastases. | |||
Nephrectomy may also be performed palliatively, for example in case of paraneoplastic syndromes. | |||
If there is metastatic disease but the metastases are available for surgical removal, studies have shown a survival benefit for complete metastasectomy. This should be considered in patients with metastatic disease. | |||
=== Thermal ablation === | |||
Thermal ablation is an alternativ to partial nephrectomy for small tumours (< 3 cm). It involves destroying the tumour with heat, radiowaves, or cold. | |||
=== Radiotherapy === | |||
Radiotherapy may be used palliatively for metastatic disease, especially for painful skeletal metastases | |||
=== Active surveillance === | |||
Active surveillance, meaning regular follow-up with imaging to look for progression and initiating curative treatment if progression occurs, is an option for small tumours, as these rarely metastasise. This should especially be considered for elderly. | |||
=== Watchful waiting === | |||
Watchful waiting refers to performing no surveillance but rather doing nothing and waiting until symptoms appear or become unbearable, at which point one can consider palliative treatment. This is especially useful for those who would not tolerate intensive cancer treatment. | |||
=== Immunotherapy === | |||
Immune checkpoint inhibitors have revolutionised treatment of metastastic RCC. They are sometimes combined with tyrisone kinase inhibitors for metastatic disease. Commonly used drugs are anti-PD-1 and anti-CTLA-4 antibodies. | |||
=== Targeted therapy === | |||
[[Category:Oncology]] | [[Category:Oncology]] | ||
[[Category:Urology]] | [[Category:Urology]] | ||
Targeted therapy may be combined with immune checkpoint inhibitors for palliative therapy of metastatic RCC. These are tyrosine kinase inhibitors like cabozanitinib, sunitinib, or lenvatinib. | |||