malignant kidney neoplasm (kidney cancer)
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Etiology
Epidemiology
- 3% of all solid tumors in men & women
- renal cell carcinoma most common renal cancer
Pathology
- arises from proximal tubule
- adenoma to carcinoma progression
- metastases
- liver (44%)
- lung (72%)
- bone (10%)
- brain (19%)
- skin (5%)
- adrenal (20%)
- ipsilateral or contralateral kidney (21%)
Genetics
- translocation involving short arm of chromosome 3
- sporadic disease
- hereditary renal cell carcinoma
- translocation involving short arm of chromosome 3
- trisomy 2 & 5
- monosomy 19
- chromosomal deletion involving UHRF2 is found in multiple kinds of malignancies
- other implicated genes WDR45, ANLN, WIPI2, LETMD1, DERPC
Clinical manifestations
- often asymptomatic until late stages
- often incidental finding during radiologic workup of unrelated medical problem
- hematuria* is most common presentation (late sign) (40%)
- fever, low grade to sporadic (20%)
- flank pain*, usually dull & constant (40%)
- palpable mass* (25%)
- weight loss (33%)
- acute varicocele in 11% of cases
- usually left-sided
- does not reduce in recumbent position
- paraneoplastic phenomena
- polycythemia (3%)
- hypertension (20%)
- amyloidosis (2%)
- neuromyopathy (3%)
* classic triad (10-20% of presentations)
Laboratory
- urinalysis: hematuria
- complete blood count (CBC)
- polycythemia (3%)
- anemia (33%)
- serum chemistries
- abnormal liver function tests (in the absence of liver metastases) (15%)
- serum calcium: hypercalcemia (5%)
- serum cortisol: elevated cortisol
- elevated erythrocyte sedimentation rate (ESR) (55%)
- serum antibodies to KCTD3 in 75% of patients
Radiology
- renal ultrasound
- abdominal CT with contrast
- magnetic resonance imaging (MRI)
- visualization of renal vein & inferior vena cava (IVC)
- tumor may invade renal vein & travel up the IVC
- staging/metastatic workup
- technetium-99m bone scan
- lung CT
- head CT if indicated
Staging
Stage 1: within capsule
Stage 2: within Gerota's fascia
Stage 3: involvement of regional lymph node, renal vein,
inferior vena cava or ipsilateral adrenal gland
Stage 4: distant metastases
also see renal cell carcinoma
Differential diagnosis
- renal abscess
- renal cyst - simple cysts generally do not cause hematuria
- angiomyolipoma
- distorted renal architecture from
- infection
- infarction
- congenital anomaly
- granuloma
- metastases to kidney
- more common than primary tumors
- rarely, clinically significant
- other tumors
- oncocytoma
- adult form of Wilm's tumor
Management
- surgery
- radical nephrectomy & regional lymphadenectomy except stage 4 tumors
- solitary metastases
- partial nephrectomy for bilateral disease
- removing the primary tumor in the setting of metastatic disease can improve overall outcome[2]
- renal cell carcinoma is radioresistant
- chemotherapy
- vinblastine (10% response rate)
- chemotherapy not very effective
- bevacizumab (Avastin) 10 mg/kg every 2 weeks for up to 1 year plus interferon alfa-2a 9 mIU 3x/week extends progression-free survival from 5-10 months[5]
- immunotherapy
- interferon-alpha
- interleukin-2 (10-20% response rate)
- lymphocyte-activated killer cells
- xenogenieic immune RNA
- bacillus Calmette-Guerin (BCG)
- hormonal therapy of limited value
- follow-up
- imaging at 1-2 months, then every 6 months for 2-5 years
- as indicated by treatment protocol
- prognosis
More general terms
More specific terms
- clear cell sarcoma of kidney
- renal cell carcinoma (hypernephroma, Grawitz tumor)
- secondary kidney cancer
- Wilm's tumor; nephroblastoma
References
- ↑ Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 560-62
- ↑ 2.0 2.1 Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 16, 18. American College of Physicians, Philadelphia 1998, 2006, 2012, 2018
- ↑ Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 521
- ↑ 4.0 4.1 4.2 Remzi M et al, Are small renal tumors harmless? Analysis of histopathologic features according to tumors 4 cm of less in diameter. J Urol 2006, 176:896 PMID: https://www.ncbi.nlm.nih.gov/pubmed/16890647
Schlomer B et al, Pathological features of renal neoplasms classified by size and symptomatology J Urol 2006, 176:1317 PMID: https://www.ncbi.nlm.nih.gov/pubmed/16952619
Matin SF et al, Residual and recurrent disease following renal energy ablative therapy: A multi-institutional study. J Urol 2006, 176:1913 PMID: https://www.ncbi.nlm.nih.gov/pubmed/17070224 - ↑ 5.0 5.1 Escudier B et al, Bevacizumab plus interferon alpha-2a for treatment of metastatic renal cell carcinoma: A randomized, double-blind phase III trial. Lancet 2007, 370:2103 PMID: https://www.ncbi.nlm.nih.gov/pubmed/18156031
- ↑ Agency for Healthcare Research and Quality (AHRQ) Executive Summary - Feb. 24, 2016 Management of Renal Masses and Localized Renal Cancer https://www.effectivehealthcare.ahrq.gov/search-for-guides-reviews-and-reports/?pageaction=displayproduct&productID=2186
- ↑ ARUP consult: Hereditary Renal Cancer Panel https://arupconsult.com/ati/hereditary-renal-cancer-panel
- ↑ National Cancer Institute Kidney (Renal Cell) Cancer - Health Professional Version https://www.cancer.gov/types/kidney/hp