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REGUB Surgery - Renal Tumours (i) (RCC intro (4% familial (AD) (VHL (3p25,…
REGUB Surgery - Renal Tumours (i)
Types
benign
adenoma (usually small, dxed incidental during imaging or @ PM)
angioma
vasc, may cause profuse haematuria
occur in young adults
often require angiography to dx them
angiomyolipoma
50% a/w tubular sclerosis (rare genetic condition that causes ending tumours to develop in different parts of body)
hamartoma
contain fat - seen on CT
main comp = haemorrhage
25% are malignant + met
oncocytoma
malignant
Wilm's tumour (nephroblastoma, in children)
Grawitz tumour (aka RCC or hypernephroma)
TCC: renal pelvis + collecting system
SCC of renal pelvis
RCC intro
3% of adult malignancies
peak in 6th-8th decade
males x2-3 more common
3% of all cancer deaths in men
most lethal of all GU neoplasms
incidence has increased x2-4% over last 3 decades
increased incidental detection
unidentified environmental risk factors
steady rise in mortality over last 3 decades
slow stage migration over last 3 decades
localised migration - 53%
regionally advanced - 20%
met disease - 22%
96% sporadic
4% familial (AD)
VHL
3p25
50% develop RCC
often bilat + multifocal
present in 3rd, 4th or 5th decade
phaeochromocytomas, renal + pancreatic cysts, cerebellar haemangioblastomas, retinal angioma, epididymal cyst adenomas
loss of both TS genes
affects transcription of hypoxia inducible factor-1 alpha - upreg of VEGF
hereditary papillary RCC - 7q34
Birt-Hog-Dubé (17p11.2, also benign skin tumours + lung cysts)
hereditary leiomyoma RCC (1q24)
adenocarc
originates in proximal convoluted tubules
tumour cells are large with clear cytoplasm (accumulation of glycogen + lipid) hence name clear cell carc
sarcomatous clear cell carc - poor prognosis
multifocal in 7-20%
RCC risk factors
largely unknown
tobacco (increases RR x1.4-2.5)
10-20% higher incidence in African Americans
acquired renal cystic disease (e.g. 1/3 of those on dialysis for 3 yrs) - increases RCC risk x3-6
nutrition (Asian migrants to western countries @ increased risk)
obesity
low socioeconomic class
asbestos
HTN
RCC spread
invading surrounding structures
direct extension to adrenal gland (7.5% in tumours >5cm)
through renal capsule
vasc invasion
a/w thrombosis
renal vein (5% @ presentation)
IVC (sub/supra-diaphragmatic)
right atrium
distant mets
lymphatics (hilar + paraaortic nodes)
haematogenous
pul 75% (cannonball mets)
bone 20%
liver 18%
adrenal
cerebral/CNS 8%
small tumours (<2cm) rarely display local invasion or distant mets
TNM
most important prognostic indicator
T1: confined to kidney, </=7cm
T1a: </= 4cm
T1b: >4cm
T2: confined to kidney, >7cm
T3: locally advance within Gerota's fascia
T3a: adrenal +/or perinephric fat
T3b: renal vein or IVC below diaphragm)
T3c: IVC above diaphragm
T4: invades beyond Gerota's fascia
N0: no regional node involvement
N1: single node
N2: >1 node
M0 or M1
Histological classification
conventional = clear cell = 70-80%
papillary = 10-15%, 40% multifocal, can be bilat
chromophobe = 5%, indolent, less aggressive
collecting duct (Bellini) = rare (<1%), young, poor prognosis
medullary cell = rare, young, black, SICKLE cell, poor prognosis, v aggressive
Clinical features
over 50% = incidental finds on imaging
classic triad in 10%: pain, mass, haematuria
haematuria in 50%
mass in 30%
met disease - 25% - bone pain, haemoptysis, oedema, weight loss, fatigue, night sweats
non-reducing acute varicocele
LL oedema
paraneoplastic syndromes
10-40%
polycythaemia (excess erythropoietin)
Fe deficient anaemia
HTN (excess renin, renal art compression)
hypercalcaemia in 10-20% (due to production of PT-like hormone)
PUO
Cushing's (ectopic ACTH)
Stauffer's syndrome: hep dysfunction - high ALP, high ESR, prolonged PT, fever, anorexia