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malignant kidney neoplasm (kidney cancer)
Etiology:
-> predisposing factors
a) tobacco
b) male sex
c) cadmium exposure
Epidemiology:
1) 3% of all solid tumors in men & women
2) renal cell carcinoma most common renal cancer
3) 2-3 times higher incidence in persons born after 1990 than in older cohort [7]
Pathology:
1) arises from proximal tubule
2) adenoma to carcinoma progression
3) metastases
a) liver (44%)
b) lung (72%)
c) bone (10%)
d) brain (19%)
e) skin (5%)
f) adrenal (20%)
g) ipsilateral or contralateral kidney (21%)
Genetics:
1) translocation involving short arm of chromosome 3
a) sporadic disease
- near von Hippel-Lindau disease locus
b) hereditary renal cell carcinoma
- translocation involving short arm of chromosome 3
2) trisomy 2 & 5
3) monosomy 19
4) chromosomal deletion involving UHRF2 is found in multiple kinds of malignancies
5) other implicated genes WDR45, ANLN, WIPI2, LETMD1, DERPC
Clinical manifestations:
1) often asymptomatic until late stages
-> often incidental finding during radiologic workup of unrelated medical problem
2) hematuria* is most common presentation (late sign) (40%)
3) fever, low grade to sporadic (20%)
4) flank pain*, usually dull & constant (40%)
5) palpable mass* (25%)
6) weight loss (33%)
6) acute varicocele in 11% of cases
a) usually left-sided
b) does not reduce in recumbent position
7) paraneoplastic phenomena
a) polycythemia (3%)
b) hypertension (20%)
c) amyloidosis (2%)
d) neuromyopathy (3%)
* classic triad (10-20% of presentations)
Laboratory:
1) urinalysis: hematuria
2) complete blood count (CBC)
a) polycythemia (3%)
b) anemia (33%)
3) serum chemistries
a) abnormal liver function tests (in the absence of liver metastases) (15%)
b) serum calcium: hypercalcemia (5%)
c) serum cortisol: elevated cortisol
4) elevated erythrocyte sedimentation rate (ESR) (55%)
5) serum antibodies to KCTD3 in 75% of patients
Radiology:
1) renal ultrasound
2) abdominal CT with contrast
3) magnetic resonance imaging (MRI)
a) visualization of renal vein & inferior vena cava (IVC)
b) tumor may invade renal vein & travel up the IVC
4) staging/metastatic workup
a) technetium-99m bone scan
b) lung CT
c) 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:
1) renal abscess
2) renal cyst - simple cysts generally do not cause hematuria
3) angiomyolipoma
4) distorted renal architecture from
a) infection
b) infarction
c) congenital anomaly
5) granuloma
6) metastases to kidney
a) more common than primary tumors
b) rarely, clinically significant
7) other tumors
a) oncocytoma
b) adult form of Wilm's tumor
Management:
1) surgery
a) radical nephrectomy & regional lymphadenectomy except stage 4 tumors
b) solitary metastases
c) partial nephrectomy for bilateral disease
d) removing the primary tumor in the setting of metastatic disease can improve overall outcome [2]
2) renal cell carcinoma is radioresistant
3) chemotherapy
a) vinblastine (10% response rate)
b) chemotherapy not very effective
c) 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]
4) immunotherapy
a) interferon-alpha
b) interleukin-2 (10-20% response rate)
c) lymphocyte-activated killer cells
d) xenogenieic immune RNA
e) bacillus Calmette-Guerin (BCG)
5) hormonal therapy of limited value
6) follow-up
a) imaging at 1-2 months, then every 6 months for 2-5 years
b) as indicated by treatment protocol
7) prognosis
a) 5 year survival 30% for non-resectable tumor
b) small (< 3 cm) tumors do often do not progress in the elderly during several years of follow-up [4],
c) observation may be an option in a subset of elderly [4]
Interactions
disease interactions
Specific
clear cell sarcoma of kidney
renal cell carcinoma (hypernephroma, Grawitz tumor)
secondary kidney cancer
Wilm's tumor; nephroblastoma
General
kidney neoplasm
urologic malignancy (urologic cancer)
References
- Saunders Manual of Medical Practice, Rakel (ed),
WB Saunders, Philadelphia, 1996, pg 560-62
- 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
- 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: 16890647
- Schlomer B et al,
Pathological features of renal neoplasms classified by size
and symptomatology
J Urol 2006, 176:1317
PMID: 16952619
- Matin SF et al,
Residual and recurrent disease following renal energy ablative
therapy: A multi-institutional study.
J Urol 2006, 176:1913
PMID: 17070224
- 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: 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
- Sung H, Chng C, Bandi P et al
Differences in cancer rates among adults born between 1920 and 1990 in the USA:
an analysis of population-based cancer registry data.
Lancet Public Health. 2024 August
PMID: 39095135 Free article.
https://www.thelancet.com/journals/lanpub/article/PIIS2468-2667(24)00156-7/fulltext
- 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