II. Epidemiology
-
Incidence
- Worldwide (2012): 337,860 new cases (9th most common cancer)
- U.S. (2013): 65,150 new cases (13,680 deaths)
- Demographics
- Most common in developed countries (partly due to incidental diagnosis on imaging)
- Male gender (twice as common as in women)
- Black men are affected more often than white men
- Diagnosis peaks at ages 60-70 years old
III. Risk Factors
-
Tobacco Use (RR>2)
- Risks increases with pack year exposure
-
Obesity (esp. women)
- Risk increases linearly with body weight
- Occupation chemical exposure
- Trichloroethylene
- Benzene
- Cadmium
- Benzidine
- Coal Tar, soot, pitch, creosote or asphalt
- Herbicides and Pesticides
- Mineral, cutting or lubricating oil
- Mustard Gas
- Pesticides
- Vinyl Chloride
- Hu (2002) Occup Med 52(3):157-64 [PubMed]
- Hereditary conditions
- Von Hippel-Lindau syndrome (via Vascular Endothelial Growth Factor)
- Hereditary papillary renal carcinoma
- Birt-Hogg-Dube syndrome
- Hereditary renal carcinoma
- Other possible risks
- NSAIDs
- Tuberous sclerosis
- Long-term renal Dialysis and Renal Transplant (via acquired cystic disease of the Kidney)
- Hypertension
IV. Types: Kidney Cancers
- Common: Tubule derived cancers (Renal Cell Carcinomas, >90% of all Kidney Cancer)
- Clear Cell Adenocarcinoma (75%)
- Clear cytoplasm in clustered cells within dense endothelial tissue
- Von Hippel-Lindau (VHL) tumor suppressor gene defect is associated in >60% of sporadic cases
- Typically diagnosed at later stage and is associated with worse prognosis
- Papillary Adenocarcinoma (15-20%)
- Basophilic cytoplasm with foamy histiocytes
- Papillary Type 1
- Associated familial syndromes: MET proto-oncogene mutation
- Papillary Type 2
- Associated familial syndromes: HLRCC syndrome (with hereditary leiomyomatosis)
- Chromophobe Adenocarcinoma of Kidney (5%)
- Empty cytoplasm and perinuclear clearing
- Lowest metastasis risk
- Associated with Chromosome loss including whole Chromosome loss (1, 2, 6, 10, 13, 17, 21)
- Associated familial syndromes: Birt-Hogg-Dube Syndrome
- Clear Cell Adenocarcinoma (75%)
- Rare (<5%): Non-tubule derived cancers (non-RCC Kidney Cancers)
- Collecting duct carcinoma
- Renal Medullary carcinoma
- Associated with Hemoglobinopathy (esp. Sickle Cell Trait)
- Urethelial carcinoma
- Variant of transitional cell carcinoma
V. Presentations
- Asymptomatic at time of diagnosis in 50% of cases (incidental finding on imaging)
- Classic presentation (Gross Hematuria, Flank Pain, palpable abdominal mass)
- Has become an uncommon presentation in U.S.
- Associated with advanced disease at time of diagnosis
- Paraneoplastic disease (20%)
- Hypertension
- Hypercalcemia
- Polycythemia
- Metastases
- Fever
- Weight loss
- Adenopathy
- Bone pain
VI. Symptoms
- Hematuria (40%)
- Flank Pain (40%)
- Weight loss (33%)
- Fever (20%)
- Hypertension (20%)
- Night Sweats
- Malaise
VII. Signs
- Palpable flank mass or abdominal mass (25%)
-
Varicocele (esp. isolated right sided or non-reducing bilateral)
- , Right Varicocele drains directly to right renal vein
- Bilateral Lower Extremity Edema
- May be caused by inferior vena cava obstruction
VIII. Labs
-
Urinalysis with microscopic exam
- See Microscopic Hematuria for evaluation protocol
IX. Imaging
- Triple-phase CT Urography
- Evaluation of persistent Microscopic Hematuria
- MRI Abdomen with and without contrast
- Consider if CT Urography is contraindicated
X. Staging: TNM System
- T Primary Tumor Staging
- TX
- Primary tumor not able to be assessed
- T0
- No evidence of primary tumor
- T1
- Tumor size <=7 cm in greatest dimension
- Tumor limited to Kidney
- T1a
- Tumor size 4 cm or less in greatest dimension
- T1b
- Tumor size 4-7 cm in greatest dimension
- T2
- Tumor size >7 cm in greatest dimension
- Tumor limited to Kidney
- T2a
- Tumor size 7-10 cm or less in greatest dimension
- T2b
- Tumor size >10 cm in greatest dimension
- T3
- T4
- Tumor spread into Gerota’s fascia (surrounding Kidney)
- Tumor spread may also occur into Adrenal Gland
- TX
- N (Node)
- NX
- Nodes not able to be assessed
- N0
- No local Lymph Node spread
- N1
- Local Lymph Node spread
- NX
- M (Metastasis)
- M0
- No spread to distant Lymph Nodes or organs
- M1
- Distant metastasis
- Spread to distant Lymph Nodes or organs (esp. lungs, bone, liver, brain)
- M0
- References
- NCI: Renal Cell Cancer Staging
XI. Staging: Grouping (AJCC)
- Stage 1: T1, N0, M0
- Tumor <=7 cm affecting a single Kidney without Lymph Node involvement or metastases
- Stage 2: T2, N0, M0
- Tumor >=7 cm affecting a single Kidney without Lymph Node involvement or metastases
- Stage 3
- T3, N0, M0
- Tumor growth into major vein or tissue around Kidney but not into Adrenal Gland or Gerota’s fascia
- T1-T3, N1, M0
- Tumor growth of any size, affecting single Kidney, without involving Gerota’s fascia
- Local Lymph Node involvement, but no distant Lymph Node involvement or metastases
- T3, N0, M0
- Stage 4
- T4, N0-1, M0
- Tumor growth into Gerota’s fascia (or into Adrenal Gland)
- Local Lymph Node involvement may occur
- No spread to distant Lymph Nodes or organs
- T1-4, N0-1, M1:
- Spread to distant Lymph Nodes or organs
- T4, N0-1, M0
XII. Management: Localized Disease
- Background
- Localized Disease accounts for 75% of RCC presentations
- Indicated if no distant metastasis
- Partial or radical nephrectomy (gold standard)
- Radical nephrectomy indications
- Solid mass >3 cm
- Complex cystic mass
- Normal contralateral Kidney
- Partial nephrectomy challenging
- Partial nephrectomy (if anatomically feasible) offers similar outcomes to radical nephrectomy
- Laparoscopic and robotic techniques are typically utilized for partial nephrectomy
- Higher rate of conversion to radical nephrectomy with laparoscopy (10%) than robotic (1%)
- Long (2012) Eur Urol 61:1257-62 [PubMed]
- Ablation (radiofrequency, microwave, cryoablation, stereotactic)
- Renal Mass biopsy (multiple core) prior to ablation for histologic diagnosis and surveillance guidance
- Used for small Renal Masses in those unable to undergo partial nephrectomy
- Limited evidence
- Active Surveillance
- Indicated in Renal Mass <2 cm in reduced Life Expectancy and high risk of surgical complication
- Renal Mass biopsy (multiple core) for histologic diagnosis and surveillance guidance
- Repeat imaging every 3-6 months
- Lymph Node dissection
- Controversial in localized disease (typically individualized based on risks and surgical findings)
- Indicated for staging in concerning Regional Lymphadenopathy
- Adrenalectomy
- Adrenal invasion is suspected
- Radical nephrectomy indications
- Other measures
- No adjuvant Chemotherapy has been found effective as of 2015
XIII. Management: Metastatic Disease (30% of cases at Renal Cell Carcinoma diagnosis)
- Cytoreductive nephrectomy (tumor debulking) AND
-
Chemotherapy: Cytokines
- Cytokines double the median survival time from 10-15 months to >24 months
- Agents (VEGF Inhibitors, Tyrosine Kinase Inhibitors, Immunotherapy)
- VEGF Receptor Blockers (e.g. Sunitinib, Pazopanib)
- mTOR Inhibitors (e.g. Everolimus, Temsirolimus)
- Immunotherapy Agents (Bevacizumab)
XIV. Prevention
- Tobacco Cessation
- Hypertension Management
- Obesity Management
- Other factors that may reduce Renal Cell Carcinoma risk
- Fatty fish intake
- Increased fruit and vegetable intake (3 or more servings per day)
- One Alcoholic beverage daily
XV. Prognosis
- Stage 1 RCC: 81-95% five year survival
- Stage 2 RCC: 74-80% five year survival
- Stage 3 RCC: 53-60% five year survival
- Stage 4 RCC: 8-10% five year survival
XVI. Resources
- Emedicine: Renal Cell Carcinoma
- NIH Kidney Cancer
Images: Related links to external sites (from Bing)
Related Studies
Definition (NCI_CDISC) | A malignant neoplasm arising from renal parenchyma. |
Definition (NCI) | A carcinoma arising from the renal parenchyma. The incidence of renal cell carcinoma has increased by 35% from 1973 to 1991. There is a strong correlation between cigarette smoking and the development of renal cell carcinoma. The clinical presentation includes : hematuria, flank pain and a palpable lumbar mass. A high percentage of renal cell carcinomas are diagnosed when an ultrasound is performed for other purposes. Diagnostic procedures include: ultra sound, intravenous pyelography and computed tomography (CT). Radical nephrectomy is the standard intervention procedure. Renal cell carcinoma is generally considered to be resistant to radiation treatment and chemotherapy. |
Definition (NCI_NCI-GLOSS) | The most common type of kidney cancer. It begins in the lining of the renal tubules in the kidney. The renal tubules filter the blood and produce urine. |
Definition (CSP) | cancerous kidney tumor composed of tubular cells; usually occurs in middle age or later in life; primary symptom is hematuria; nephrectomy is usually necessary. |
Definition (MSH) | A heterogeneous group of sporadic or hereditary carcinoma derived from cells of the KIDNEYS. There are several subtypes including the clear cells, the papillary, the chromophobe, the collecting duct, the spindle cells (sarcomatoid), or mixed cell-type carcinoma. |
Concepts | Neoplastic Process (T191) |
MSH | D002292 |
ICD10 | M8312/3 |
SnomedCT | 309511000009104, 254915003, 702391001, 41607009 |
English | Adenocarcinoma, Renal Cell, Adenocarcinomas, Renal Cell, Carcinoma, Nephroid, Carcinoma, Renal Cell, Carcinomas, Nephroid, Carcinomas, Renal Cell, Nephroid Carcinoma, Nephroid Carcinomas, Renal Cell Adenocarcinomas, Renal Cell Carcinomas, RENAL CELL CARCINOMA, Renal cell carcinoma-morpholog, Renal carcinoma, adenocarcinoma of kidney, adenocarcinoma of kidney (diagnosis), Renal cell carcinoma stage unspecified, Renal cell cancer, Renal cell carcinoma NOS, renal cell carcinoma (diagnosis), renal cell carcinoma, renal adenocarcinoma, Carcinoma, Renal Cell [Disease/Finding], Renal Cell Cancer, Cancers, Renal Cell, Cancer, Renal Cell, Renal Cell Cancers, adenocarcinoma renal, hypernephroid carcinomas, kidney adenocarcinoma, kidney carcinoma, Carcinoma;kidney, carcinoma of kidney, rccs, cancer cells renal, carcinoma cell renal, of kidney carcinoma, adenocarcinoma cells renal, carcinomas renal, grawitz tumor, carcinoma cells renal, rcc, carcinoma kidney, carcinoma renal, cell renal cancer, adenocarcinoma kidneys, cancer cell renal, hypernephroma, Renal Cell Carcinoma, Papillary, Adenocarcinoma, Renal, Adenocarcinoma Of Kidney, Grawitz tumor, Grawitz tumour, Adenocarcinoma of kidney (disorder), Renal adenocarcinoma, Adenocarcinoma of kidney, ADENOCARCINOMA OF THE KIDNEY, CARCINOMA, RENAL CELL, MALIGNANT, RENAL CELL CARCINOMA, STAGE UNSPECIFIED, RENAL CELL CANCER, RCC, KIDNEY ADENOCARCINOMA, ADENOCARCINOMA OF KIDNEY, RENAL CELL ADENOCARCINOMA, Renal cell carcinoma, NOS, Renal cell carcinoma (disorder), Renal cell carcinoma, Renal cell adenocarcinoma, Renal cell carcinoma - morphology, Renal cell carcinoma (morphologic abnormality), renal cell cancer, renal cell adenocarcinoma, adenocarcinoma of the kidney, kidney cancer, renal cell adenocarcinoma, Kidney (renal cell) cancer, carcinoma; renal cell, adenocarcinoma; renal cell, renal cell; adenocarcinoma, renal cell; carcinoma, Renal Cell Carcinoma, Renal Cell Adenocarcinoma, Adenocarcinoma of Kidney, Adenocarcinoma of the Kidney, Kidney Adenocarcinoma, Renal Cell Carcinoma, Stage Unspecified, renal carcinoma |
Swedish | Njurcellskarcinom |
Finnish | Munuaissolukarsinooma |
Italian | Adenocarcinoma a cellule renali, Carcinoma renale, Cancro a cellule renali, Carcinoma a cellule renali NAS, Adenocarcinoma del rene, Carcinoma a cellule renali, stadio non specificato, Tumore di Grawitz, Carcinoma a cellule renali |
Russian | GIPERNEFROMA, KARTSINOMA POCHECHNO-KLETOCHNAIA, GRAVITTSA OPUKHOL', KARTSINOMA GIPERNEFROIDNAIA, ADENOKARTSINOMA POCHECHNO-KLETOCHNAIA, АДЕНОКАРЦИНОМА ПОЧЕЧНО-КЛЕТОЧНАЯ, ГИПЕРНЕФРОМА, ГРАВИТЦА ОПУХОЛЬ, КАРЦИНОМА ГИПЕРНЕФРОИДНАЯ, КАРЦИНОМА ПОЧЕЧНО-КЛЕТОЧНАЯ |
Spanish | Adenocarcinoma de riñón, Adenocarcinoma de células renales, Cáncer de células renales, Carcinoma de células renales, estadio no especificado, Carcinoma de células renales, Carcinoma de células renales NEOM, Tumor de Grawitz, adenocarcinoma de células renales - morfología, adenocarcinoma de células renales, adenocarcinoma de riñón, carcinoma de células renales (anomalía morfológica), carcinoma de células renales, Adenocarcinoma de Células Renales, Carcinoma de Células Renales |
French | Adénocarcinome à cellules rénales, Carcinome à cellules rénales SAI, Adénocarcinome du rein, Carcinome rénal stade non précisé, Cancer à cellules rénales, Carcinome à cellules rénales, Tumeur de Grawitz, Néphrocarcinome, Carcinome du rein, Carcinome rénal |
Dutch | niercel carcinoom, adenocarcinoom van de nier, nierceladenocarcinoom, niercelcarcinoom stadium niet-gespecificeerd, niercelkanker, niercelcarcinoom NAO, niercelcarcinoom, adenocarcinoom; niercel, carcinoom; niercel, niercel; adenocarcinoom, niercel; carcinoom, Adenocarcinoom, niercel-, Carcinoom, niercel-, Grawitz-tumor, Hypernefroom, Niercelcarcinoom, Nierkanker, Carcinoom, hypernefroïd |
German | Nierenzelladenokarzinom, Nierenzellkrebs, Adenokarzinom der Niere, Nierenzellkarzinom NNB, Nierenzellkarzinom unbekannten Stadiums, Grawitz-Tumor, Adenokarzinom, Nierenzell-, Karzinom, Nierenzell-, Nephroides Karzinom, Nierenzellkarzinom |
Portuguese | Cancro de células renais NE, Adenocarcinoma do rim, Carcinoma de células renais estádio não especificado, Cancro de células renais, Adenocarcinoma de células renais, Carcinoma de células renais, Tumor de Grawitz, Adenocarcinoma de Células Renais, Carcinoma de Células Renais |
Japanese | 腎細胞癌、病期不明, 腎細胞癌NOS, ジンセンガン, ジンサイボウガンNOS, ジンサイボウガンビョウキフメイ, ジンサイボウガン, Grawitz腫瘍, 腎細胞癌, グラヴィッツシュヨウ, グラヴィッツ腫瘍, 副腎腫, 腎細胞腺癌, 腎腺癌, グラーヴィッツ腫瘍, 癌-腎細胞, 腎癌(腎細胞癌), 腺癌-腎細胞 |
Czech | Adenokarcinom ledviny, Rakovina ledvinových buněk, Karcinom ledvinové buňky, Karcinom ledviny stadium blíže neurčené, Karcinom ledviny NOS, Grawitzův tumor, Grawitzův nádor, karcinom z renálních buněk, papilární renální karcinom, adenokarcinom ledviny, karcinom z ledvinových buněk |
Croatian | KARCINOM BUBREŽNIH STANICA |
Polish | Guz Grawitza, Rak jasnokomórkowy nerki, Rak nerki miąższowokomórkowy, Rak nerkowokomórkowy, Gruczolakorak nerki, Hipernefroma |
Hungarian | Vesesejt-adenocarcinoma, Vesesejtes carcinoma k.m.n., Renális sejt carcinoma, Vese adenocarcinomája, Vesesejtes rák, Vesesejt nem meghatározott stádiumú carcinoma, Grawitz-tumor |
Norwegian | Nyrecellekarsinom |
Ontology: Conventional (Clear Cell) Renal Cell Carcinoma (C0279702)
Definition (NCI_NCI-GLOSS) | The most common type of kidney cancer. It begins in the lining of the renal tubules in the kidney. The renal tubules filter the blood and produce urine. |
Definition (NCI) | A malignant epithelial neoplasm of the kidney characterized by the presence of lipid-containing clear cells within a vascular network. The tumor may metastasize to unusual sites and late metastasis is common. |
Concepts | Neoplastic Process (T191) |
MSH | D002292 |
SnomedCT | 254915003, 188251003, 41607009 |
English | HYPERNEPHROMA, hypernephroid carcinoma, clear cell adenocarcinoma of kidney, clear cell adenocarcinoma of kidney (diagnosis), clear cell renal carcinoma, clear cell carcinoma of kidney, carcinoma cell clear kidney, Clear cell carcinoma of the kidney, Clear cell renal cell carcinoma, Renal clear cell carcinoma, RCC, clear cell adenocarcinoma, Renal cell carcinoma, clear cell adenocarcinoma, Grawitz tumor, Clear cell carcinoma of kidney, Grawitz tumour, Clear cell carcinoma of kidney (disorder), Hypernephroma (disorder), hypernephroma, renal clear cell carcinoma, carcinoma, clear cell, renal, clear cell carcinoma of the kidney, kidney cancer, clear cell carcinoma, Grawitz; tumor, tumor; Grawitz, Clear Cell Adenocarcinoma of Kidney, Clear Cell Adenocarcinoma of the Kidney, Clear Cell Adenocarcinoma, Kidney, Clear Cell Carcinoma of Kidney, Clear Cell Carcinoma of the Kidney, Conventional (Clear Cell) Renal Cell Adenocarcinoma, Conventional Renal Cell Carcinoma, Kidney Clear Cell Adenocarcinoma, Kidney Clear Cell Carcinoma, Renal Clear Cell Adenocarcinoma, Renal Clear Cell Carcinoma, Carcinoma, Hypernephroid, Carcinomas, Hypernephroid, Grawitz Tumor, Hypernephroid Carcinoma, Hypernephroid Carcinomas, Hypernephroma, Hypernephromas, Tumor, Grawitz, Clear Cell Renal Cell Carcinoma, Clear Cell Renal Carcinoma, Conventional (Clear Cell) Renal Cell Carcinoma |
French | Tumeur de Grawitz, Carcinome à cellules claires rénal, Carcinome à cellules rénales, à cellules claires, Adénocarcinome rénal à cellules claires, Hypernéphrome, Épithelioma du rein à cellules claires, Épithelioma rénal à cellules claires, Carcinome du rein à cellules claires |
German | Klarzelliges Nierenzellkarzinom, Klarzellkarzinom der Nierenzellen, Klarzellkarzinom der Niere, Grawitz-Tumor, Hypernephrom, Karzinom, hypernephroides, Klarzelliges Nierenkarzinom |
Dutch | hypernefroom, clear cell carcinoom van de nier, clear cell niercelcarcinoom, nier clear cellcarcinoom, Grawitz; tumor, tumor; Grawitz |
Japanese | 副腎腫, フクジンシュ, 腎明細胞癌, ジンメイサイボウガン |
Czech | Hypernefrom, Grawitzův nádor, světlobuněčný renální karcinom, Karcinom ledviny z vodojasných buněk, Karcinom ledviny ze světlých buněk |
Hungarian | Hypernephroma, Vese clear cell carcinoma, Renalis világos-sejtes carcinoma, Tisztasejtes vesesejt-carcinoma |
Norwegian | Klarcellet nyrecellekarsinom |
Portuguese | Carcinoma Hipernefroide, Carcinoma renal de células claras, Carcinoma de células renais variante de células claras, Hipernefroma, Tumor de Grawitz |
Italian | Carcinoma a cellule chiare del rene, Carcinoma del rene a cellule chiare, Carcinoma renale a cellule chiare, Ipernefroma, Tumore di Grawitz, Carcinoma ipernefroide |
Spanish | Carcinoma renal de células claras, Carcinoma de células renales variante de células claras, Carcinoma de células claras del riñón, carcinoma de células claras de riñón (trastorno), carcinoma de células claras de riñón, hipernefroma (trastorno), hipernefroma, tumor de Grawitz, Hipernefroma, Carcinoma Hipernefroide, Tumor de Grawitz |
Ontology: Chromophobe Renal Cell Carcinoma (C1266042)
Definition (NCI) | A type of carcinoma that comprises a minority of renal cell carcinomas. It is characterized by loss of chromosomes 1 and Y. Based on the cytoplasmic characteristics of the neoplastic cells, this type of carcinoma is classified as classic (typical) or eosinophilic. It has a much better prognosis than other renal cell carcinomas. |
Concepts | Neoplastic Process (T191) |
MSH | D002292 |
ICD10 | M8270/3 |
SnomedCT | 1443001, 128667008 |
French | Carcinome rénal à cellules chromophobes |
German | Chromophobes Nierenzellkarzinom |
English | RENAL CELL CARCINOMA, CHROMOPHOBE, Chromophobe adenocarcinoma, Chromophobe carcinoma, Chromophobe carcinoma (morphologic abnormality), Chromophobe cell renal carcinoma, Renal cell carcinoma, chromophobe cell (morphologic abnormality), Renal cell carcinoma, chromophobe cell, Chromophobe Adenocarcinoma, Chromophobe Carcinoma of Kidney, Chromophobe Carcinoma of the Kidney, Chromophobe Carcinoma, Chromophobe Cell Carcinoma of Kidney, Chromophobe Cell Carcinoma of the Kidney, Renal Cell Carcinoma, Chromophobe Type, Chromophobe Renal Cell Carcinoma |
Czech | chromofobní renální karcinom |
Norwegian | Kromofobt nyrecellekarsinom |
Italian | Carcinoma a cellule renali cromofobo |
Spanish | adenocarcinoma cromófobo, carcinoma cromófobo (anomalía morfológica), carcinoma cromófobo, carcinoma de células renales, células cromófobas (anomalía morfológica), carcinoma de células renales, células cromófobas, carcinoma renal de células cromófobas |
Ontology: Papillary Renal Cell Carcinoma (C1306837)
Definition (NCI) | Also known as chromophil carcinoma, it represents a minority of renal cell carcinomas. It can be hereditary or sporadic. The sporadic papillary renal cell carcinoma is characterized by trisomy of chromosomes 7, 16, and 17, and loss of chromosome Y. The peak incidence is in the sixth and seven decades. It is classified as type 1 or 2, based on the cytoplasmic volume and the thickness of the lining neoplastic cells. The prognosis is more favorable than for conventional (clear cell) renal cell carcinoma. -- 2003 |
Concepts | Neoplastic Process (T191) |
MSH | D002292 |
SnomedCT | 4797003 |
French | Carcinome papillaire du rein |
German | Papilläres Nierenzellkarzinom |
English | Papillary Renal Cell Carcinoma, papillary renal cell carcinoma, Chromophil Renal Cell Carcinoma, Papillary renal cell carcinoma, Chromophil Carcinoma of Kidney, Chromophil Carcinoma of the Kidney, Papillary (Chromophil) Renal Cell Carcinoma |
Norwegian | Papillært nyrecellekarsinom |
Italian | Carcinoma papillare a cellule renali |