Urology Book

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Renal Cell Carcinoma

Aka: Renal Cell Carcinoma, Kidney Cancer, Renal Cancer, Renal Cell Adenocarcinoma, Clear Cell Adenocarcinoma of Kidney, Papillary Adenocarcinoma of Kidney, Chromophobe Adenocarcinoma of Kidney
  1. Epidemiology
    1. Incidence
      1. Worldwide (2012): 337,860 new cases (9th most common cancer)
      2. U.S. (2013): 65,150 new cases (13,680 deaths)
    2. Demographics
      1. Most common in developed countries (partly due to incidental diagnosis on imaging)
  2. Risk Factors
    1. Tobacco Use (RR>2)
      1. Risks increases with pack year exposure
    2. Obesity (esp. women)
      1. Risk increases linearly with body weight
    3. Occupation chemical exposure
      1. Trichloroethylene
      2. Benzene
      3. Cadmium
      4. Benzidine
      5. Coal tar, soot, pitch, creosote or asphalt
      6. Herbicides and Pesticides
      7. Mineral, cutting or lubricating oil
      8. Mustard Gas
      9. Pesticides
      10. Vinyl chloride
      11. Hu (2002) Occup Med 52(3):157-64 [PubMed]
    4. Hereditary conditions
      1. Von Hippel-Lindau syndrome
      2. Hereditary papillary renal carcinoma
      3. Birt-Hogg-Dube syndrome
      4. Hereditary renal carcinoma
    5. Other possible risks
      1. NSAIDs
      2. Tuberous sclerosis
      3. Long-term renal Dialysis and renal transplant (via acquired cystic disease of the Kidney)
  3. Types: Kidney Cancers
    1. Common: Tubule derived cancers (Renal Cell Carcinomas)
      1. Clear Cell Adenocarcinoma (70%)
        1. Clear cytoplasm in clustered cells within dense endothelial tissue
        2. Von Hippel-Lindau (VHL) tumor suppressor gene defect is associated in >60% of sporadic cases
      2. Papillary Adenocarcinoma (second most common)
        1. Basophilic cytoplasm with foamy histiocytes
        2. Papillary Type 1
          1. Associated familial syndromes: MET proto-oncogene mutation
        3. Papillary Type 2
          1. Associated familial syndromes: HLRCC syndrome (with hereditary leiomyomatosis)
      3. Chromophobe Adenocarcinoma of Kidney
        1. Empty cytoplasm and perinuclear clearing
        2. Lowest metastasis risk
        3. Associated with Chromosome loss including whole Chromosome loss (1, 2, 6, 10, 13, 17, 21)
        4. Associated familial syndromes: Birt-Hogg-Dube Syndrome
    2. Rare (<5%): Non-tubule derived cancers (non-RCC Kidney Cancers)
      1. Collecting duct carcinoma
      2. Renal Medullary carcinoma
        1. Associated with Hemoglobinopathy (esp. Sickle Cell Trait)
      3. Urethelial carcinoma
        1. Variant of transitional cell carcinoma
  4. Symptoms
    1. Hematuria (40%)
    2. Flank Pain (40%)
    3. Weight loss (33%)
    4. Fever (20%)
    5. Hypertension (20%)
    6. Night Sweats
    7. Malaise
  5. Signs
    1. Palpable flank mass or abdominal mass (25%)
    2. Varicocele (esp. left side)
  6. Staging: TNM System
    1. T Primary Tumor Staging
      1. TX
        1. Primary tumor not able to be assessed
      2. T0
        1. No evidence of primary tumor
      3. T1
        1. Tumor size <=7 cm in greatest dimension
        2. Tumor limited to Kidney
        3. T1a
          1. Tumor size 4 cm or less in greatest dimension
        4. T1b
          1. Tumor size 4-7 cm in greatest dimension
      4. T2
        1. Tumor size >7 cm in greatest dimension
        2. Tumor limited to Kidney
        3. T2a
          1. Tumor size 7-10 cm or less in greatest dimension
        4. T2b
          1. Tumor size >10 cm in greatest dimension
      5. T3
        1. Tumor local spread
          1. No growth into Gerota’s fascia (surrounding Kidney)
        2. T3a
          1. Growth into major vein or fatty tissue around Kidney
        3. T3b
          1. Growth into abdominal vena cava
        4. T3c
          1. Growth into chest vena cava or the vena cava wall
      6. T4
        1. Tumor spread into Gerota’s fascia (surrounding Kidney)
        2. Tumor spread may also occur into Adrenal Gland
    2. N (Node)
      1. NX
        1. Nodes not able to be assessed
      2. N0
        1. No local lymph node spread
      3. N1
        1. Local lymph node spread
    3. M (Metastasis)
      1. M0
        1. No spread to distant lymph nodes or organs
      2. M1
        1. Distant metastasis
        2. Spread to distant lymph nodes or organs (esp. lungs, bone, liver, brain)
    4. References
      1. NCI: Renal Cell Cancer Staging
        1. http://www.cancer.gov/types/kidney/hp/kidney-treatment-pdq#section/_7
  7. Staging: Grouping (AJCC)
    1. Stage 1: T1, N0, M0
      1. Tumor <=7 cm affecting a single Kidney without lymph node involvement or metastases
    2. Stage 2: T2, N0, M0
      1. Tumor >=7 cm affecting a single Kidney without lymph node involvement or metastases
    3. Stage 3
      1. T3, N0, M0
        1. Tumor growth into major vein or tissue around Kidney but not into Adrenal Gland or Gerota’s fascia
      2. T1-T3, N1, M0
        1. Tumor growth of any size, affecting single Kidney, without involving Gerota’s fascia
        2. Local lymph node involvement, but no distant lymph node involvement or metastases
    4. Stage 4
      1. T4, N0-1, M0
        1. Tumor growth into Gerota’s fascia (or into Adrenal Gland)
        2. Local lymph node involvement may occur
        3. No spread to distant lymph nodes or organs
      2. T1-4, N0-1, M1:
        1. Spread to distant lymph nodes or organs
  8. Management: Localized Disease
    1. Background
      1. Localized Disease accounts for 75% of RCC presentations
      2. Indicated if no distant metastasis
    2. Partial or radical nephrectomy (gold standard)
      1. Partial nephrectomy (if anatomically feasible) offers similar outcomes to radical nephrectomy
        1. Jonasch (2012) Mol Cancer Res 10:859-80 [PubMed]
      2. Laparoscopic and robotic techniques are typically utilized for partial nephrectomy
        1. Higher rate of conversion to radical nephrectomy with laparoscopy (10%) than robotic (1%)
        2. Long (2012) Eur Urol 61:1257-62 [PubMed]
      3. Ablation (radiofrequency, microwave, cryoablation, stereotactic)
        1. Used for small Renal Masses in those unable to undergo partial nephrectomy
        2. Limited evidence
      4. Lymph node dissection
        1. Controversial in localized disease (typically individualized based on risks and surgical findings)
    3. Other measures
      1. No adjuvant Chemotherapy has been found effective as of 2015
  9. Management: Metastatic Disease
    1. Cytoreductive nephrectomy (tumor debulking) AND
    2. Chemotherapy: Cytokines
      1. Cytokines double the median survival time from 10-15 months to >24 months
      2. Agents
        1. VEGF Receptor Blockers (e.g. sunitinib, pazopanib)
        2. mTOR Inhibitors (e.g. Everolimus, temsirolimus)
  10. Prognosis
    1. Stage 1 RCC: 80-95% five year survival
    2. Stage 2 RCC: 80% five year survival
    3. Stage 3 RCC: 60% five year survival
    4. Stage 4 RCC: 10% five year survival
  11. Resources
    1. Emedicine: Renal Cell Carcinoma
      1. http://emedicine.medscape.com/article/281340-clinical#showall
    2. NIH Kidney Cancer
      1. http://www.cancer.gov/types/kidney/hp
  12. References
    1. Jonasch (2014) BMJ 349:g4797 +PMID:25385470 [PubMed]

Renal Cell Carcinoma (C0007134)

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
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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