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{{Renal cell carcinoma}}
{{Renal cell carcinoma}}
==Overview==


==Overview==
==Historical Perspective==
'''Renal cell carcinoma''' is the most common form of '''kidney cancer''' arising from the [[renal tubule]]. It is the most common type of [[kidney]] [[cancer]] in adults. Initial treatment is [[surgery]]. It is notoriously resistant to [[radiation therapy]] and [[chemotherapy]], although some cases respond to [[immunotherapy]]. The advent of targeted cancer therapies such as [[sunitinib]] has vastly improved the outlook for treatment of RCC.
Renal cell carcinoma was first described in 1826. Following contradictory hypotheses regarding the origin of [[renal]] [[tumors]], it was not until 1960 that Oberling and colleagues showed that renal carcinomas originate from renal cells.


==Classification==
==Classification==
Classification of renal cell carcinomas according to histopathological subtypes is based on the 1997 classification by Heidelberg and colleagues. Classification may also be according to the stage of renal cell carcinoma, which often is based on Robson classification system or the tumor-lymph node-metastasis (TNM) system.
==Pathophysiology==
The pathophysiology of renal cell carcinomas plays an important role in differentiating different types of renal cell carcinomas and in choosing appropriate targeted medical therapies. Sporadic forms of clear cell renal carcinomas, the most common form of renal cell carcinomas, have similar pathophysiological mechanisms to those of von Hippel Lindau (VHL) disease. On the other hand, ''MET'' proto-oncogene seems to play a role in the disease pathogenesis of papillary forms of renal cell carcinoma. Uniquely also, oncocytomas are benign tumors that arise from type A intercalated cells, whereas chromophobe renal cell carcinoma arises from type B intercalated cells.
==Causes==
The causes of renal cell carcinoma include [[VHL syndrome|von hippel-lindau]] ([[Von Hippel-Lindau disease|VHL]]), hereditary [[paragangliomas]], [[leiomyomatosis]], [[Birt-Hogg-Dube syndrome|birt-hogg-dube syndrome]], and several other genetic factors.
==Differentiating Renal cell carcinoma from other Diseases==
The differential diagnosis of renal cell carcinomas includes metastastic disease, cysts, abscesses, lymphomas, and other benign and malignant tumors, and associated syndromes.
==Epidemiology and Demographics==
In 2011, the age-adjusted [[prevalence]] of kidney cancer was 85.9 per 100,000 in the United States, the [[incidence]] was 15.28 per 100,000 persons. The male to female ratio is approximately 2 to 1. The median age of presentation is typically 60 years old.
==Risk Factors==
Common risk factors of renal cell carcinoma include cigarette smoking, [[obesity]], [[hypertension]], end-stage [[renal failure]], acquired cystic renal disease, [[acetaminophen]] and analgesic drug use, [[asbestos]] or trichloroethylene exposure, [[tuberous sclerosis]], von-Hippel Lindau, hereditary [[paraganglioma]], [[leiomyomatosis]], and [[Birt-Hogg-Dube syndrome|birt-hogg-dube syndrome]].
==Screening==
There are currently no guidelines for screening for renal cell carcinoma.
==Natural History, Complications and Prognosis==
Common complications of renal cell carcinoma include [[hypertension]], [[hypercalcemia]], [[Budd-Chiari syndrome|budd-chiari syndrome]], [[hepatic vein thrombosis]], [[polycythemia]], [[renal failure]], [[metastasis]]. Prognosis is generally poor, and the 5-year mortality of renal cell carcinoma is approximately 73.2%.
==Diagnosis==
===Diagnostic study of choice===
A needle biopsy should always be performed when the finding of a renal mass is detected on imaging.
===History and Symptoms===
Common symptoms of renal cell carcinoma include [[hematuria]], [[flank pain]], palpable [[abdominal mass]],[[weight loss]] and [[anorexia]].
===Physical Examination===
Common physical examination findings of renal cell carcinoma include low [[body mass index]], high [[blood pressure]], palpation of [[abdominal mass]], auscultation of [[abdominal bruit]], [[varicocele]], [[muscle atrophy]], skin [[pallor]], facial [[flushing]], and supraclavicular [[lymphadenopathy]].
===Laboratory Findings===
Laboratory findings of renal cell carcinoma include [[anemia]], [[polycythemia]], [[hypercalcemia]], elevated [[ESR]], elevated [[liver function tests]], elevated [[alkaline phosphatase]], elevated [[lactate dehydrogenase]], elevated serum [[creatinine]], [[hematuria]], and [[cancer cells]] on urine cytology.
===Electrocardiogram===
There are no ECG findings associated with renal cell carcinoma.
===X-ray===
There are no x-ray findings associated with renal cell carcinoma. However, an x-ray may be helpful in the diagnosis of [[pulmonary metastasis]] of renal cell carcinoma.
===Ultrasound===
[[Ultrasound]] may be helpful in the diagnosis of renal cell carcinoma. [[Ultrasound]] may be helpful when CT scan results are equivocal.
===CT scan===
Renal CT scan may be helpful in the diagnosis of renal cell carcinoma. CT may be used to detect [[neoplastic]] masses that may define renal cell carcinoma or [[metastasis]] of primary cancer.
===MRI===
Renal MRI may be helpful in the diagnosis of renal cell carcinoma. Due to the use of IV [[gadolinium]] in MRI, the risk of nephrogenic systemic [[fibrosis]] (NSF) must always be considered.
===Other Imaging Findings===
PET CT and [[angiography]] may be helpful in the diagnosis or following-up patients with renal cell carcinoma. [[Bone scan]] is recommended in patients with elevated [[alkaline phosphatase]] to rule out [[bone metastasis]].
===Other Diagnostic Studies===
There are no other diagnostic studies associated with renal cell carcinoma.
==Treatment==
===Medical treatment===
The medical therapies of renal cell carcinoma include [[chemotherapy]], [[hormone treatment]], [[immunotherapy]], and [[targeted therapy]].
===Surgery===
Surgery is the mainstay of treatment for renal cell carcinoma. Partial [[nephrectomy]] and ablation are recommended for patients who develop small renal masses. For patients with locally advanced renal tumors, radical [[nephrectomy]] is recommended. Moreover, radical [[nephrectomy]] or organ sparing treatment, along with the use of [[interferon alfa]], is also used as part of a more aggressive management plan in cases with metastatic renal tumors.
===Primary prevention===
Primary prevention strategies include early [[screening]], changing lifestyle, decreasing exposure to occupational risk factors, and controlling of [[hypertension]].
===Secondary prevention===
There are no established measures for the secondary prevention of renal cell carcinoma.


Recent genetic studies have altered the approaches used in classifying renal cell carcinoma. The following system can be used to classify these tumors:<ref name="pmid10768592">{{cite journal |author=Reuter VE, Presti JC |title=Contemporary approach to the classification of renal epithelial tumors |journal=Semin. Oncol. |volume=27 |issue=2 |pages=124–37 |year=2000 |month=April |pmid=10768592 |doi= |url=}}</ref><ref name="pmid12351585">{{cite journal |author=Bodmer D, van den Hurk W, van Groningen JJ, ''et al'' |title=Understanding familial and non-familial renal cell cancer |journal=Hum. Mol. Genet. |volume=11 |issue=20 |pages=2489–98 |year=2002 |month=October |pmid=12351585 |doi= |url=http://hmg.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=12351585}}</ref><ref name="isbn0-7216-0187-1">{{cite book |author=Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. |title=Robbins and Cotran pathologic basis of disease |publisher=Elsevier Saunders |location=St. Louis, Mo |year=2005 |pages=1016 |isbn=0-7216-0187-1 |oclc= |doi= |accessdate=}}</ref>


* [[Clear cell carcinoma]] ([[Von Hippel-Lindau tumor suppressor|VHL]] and others on [[chromosome 3]])
* [[Papillary]] carcinoma ([[C-Met|MET]], [[PRCC (gene)|PRCC]])
* [[Chromophobe]] renal carcinoma
* [[Collecting duct]] carcinoma


Other associated genes include TRC8, OGG1, [[HNF1A]], [[HNF1B]], [[TFE3]], RCCP3, and RCC17.
[[Category:Kidney diseases]]
[[Category:Types of cancer]]
[[Category:Nephrology]]
[[Category:Mature chapter]]


==References==
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[[Category:Nephrology]]
[[Category:Surgery]]

Latest revision as of 16:33, 9 October 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Faizan Sheraz, M.D. [2]

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Overview

Historical Perspective

Renal cell carcinoma was first described in 1826. Following contradictory hypotheses regarding the origin of renal tumors, it was not until 1960 that Oberling and colleagues showed that renal carcinomas originate from renal cells.

Classification

Classification of renal cell carcinomas according to histopathological subtypes is based on the 1997 classification by Heidelberg and colleagues. Classification may also be according to the stage of renal cell carcinoma, which often is based on Robson classification system or the tumor-lymph node-metastasis (TNM) system.

Pathophysiology

The pathophysiology of renal cell carcinomas plays an important role in differentiating different types of renal cell carcinomas and in choosing appropriate targeted medical therapies. Sporadic forms of clear cell renal carcinomas, the most common form of renal cell carcinomas, have similar pathophysiological mechanisms to those of von Hippel Lindau (VHL) disease. On the other hand, MET proto-oncogene seems to play a role in the disease pathogenesis of papillary forms of renal cell carcinoma. Uniquely also, oncocytomas are benign tumors that arise from type A intercalated cells, whereas chromophobe renal cell carcinoma arises from type B intercalated cells.

Causes

The causes of renal cell carcinoma include von hippel-lindau (VHL), hereditary paragangliomas, leiomyomatosis, birt-hogg-dube syndrome, and several other genetic factors.

Differentiating Renal cell carcinoma from other Diseases

The differential diagnosis of renal cell carcinomas includes metastastic disease, cysts, abscesses, lymphomas, and other benign and malignant tumors, and associated syndromes.

Epidemiology and Demographics

In 2011, the age-adjusted prevalence of kidney cancer was 85.9 per 100,000 in the United States, the incidence was 15.28 per 100,000 persons. The male to female ratio is approximately 2 to 1. The median age of presentation is typically 60 years old.

Risk Factors

Common risk factors of renal cell carcinoma include cigarette smoking, obesity, hypertension, end-stage renal failure, acquired cystic renal disease, acetaminophen and analgesic drug use, asbestos or trichloroethylene exposure, tuberous sclerosis, von-Hippel Lindau, hereditary paraganglioma, leiomyomatosis, and birt-hogg-dube syndrome.

Screening

There are currently no guidelines for screening for renal cell carcinoma.

Natural History, Complications and Prognosis

Common complications of renal cell carcinoma include hypertension, hypercalcemia, budd-chiari syndrome, hepatic vein thrombosis, polycythemia, renal failure, metastasis. Prognosis is generally poor, and the 5-year mortality of renal cell carcinoma is approximately 73.2%.

Diagnosis

Diagnostic study of choice

A needle biopsy should always be performed when the finding of a renal mass is detected on imaging.

History and Symptoms

Common symptoms of renal cell carcinoma include hematuria, flank pain, palpable abdominal mass,weight loss and anorexia.

Physical Examination

Common physical examination findings of renal cell carcinoma include low body mass index, high blood pressure, palpation of abdominal mass, auscultation of abdominal bruit, varicocele, muscle atrophy, skin pallor, facial flushing, and supraclavicular lymphadenopathy.

Laboratory Findings

Laboratory findings of renal cell carcinoma include anemia, polycythemia, hypercalcemia, elevated ESR, elevated liver function tests, elevated alkaline phosphatase, elevated lactate dehydrogenase, elevated serum creatinine, hematuria, and cancer cells on urine cytology.

Electrocardiogram

There are no ECG findings associated with renal cell carcinoma.

X-ray

There are no x-ray findings associated with renal cell carcinoma. However, an x-ray may be helpful in the diagnosis of pulmonary metastasis of renal cell carcinoma.

Ultrasound

Ultrasound may be helpful in the diagnosis of renal cell carcinoma. Ultrasound may be helpful when CT scan results are equivocal.

CT scan

Renal CT scan may be helpful in the diagnosis of renal cell carcinoma. CT may be used to detect neoplastic masses that may define renal cell carcinoma or metastasis of primary cancer.

MRI

Renal MRI may be helpful in the diagnosis of renal cell carcinoma. Due to the use of IV gadolinium in MRI, the risk of nephrogenic systemic fibrosis (NSF) must always be considered.

Other Imaging Findings

PET CT and angiography may be helpful in the diagnosis or following-up patients with renal cell carcinoma. Bone scan is recommended in patients with elevated alkaline phosphatase to rule out bone metastasis.

Other Diagnostic Studies

There are no other diagnostic studies associated with renal cell carcinoma.

Treatment

Medical treatment

The medical therapies of renal cell carcinoma include chemotherapy, hormone treatment, immunotherapy, and targeted therapy.

Surgery

Surgery is the mainstay of treatment for renal cell carcinoma. Partial nephrectomy and ablation are recommended for patients who develop small renal masses. For patients with locally advanced renal tumors, radical nephrectomy is recommended. Moreover, radical nephrectomy or organ sparing treatment, along with the use of interferon alfa, is also used as part of a more aggressive management plan in cases with metastatic renal tumors.

Primary prevention

Primary prevention strategies include early screening, changing lifestyle, decreasing exposure to occupational risk factors, and controlling of hypertension.

Secondary prevention

There are no established measures for the secondary prevention of renal cell carcinoma.


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