RCC RENAL CELL CARCINOMA Also called hypernephroma & Grawitz tumor Just like most other urological

malignancies this is more common in men It is more common in the upper lobes The classical triad of hematuria , flank , flank mass is seen in only 10 % of the cases Most of these are adeno carcinomas The cell of origin is the PCT The sub types are 1. 2. 3. 4. clear cell type (the most common ; 3p deletion is characteristic) papillary cell type (MET proto oncogene mutations , chr 7 &17 inolvement is typical ) oncocytomas (these typically have the central stellate scar and spoke wheel appearance) chromophobe type (show hypo diploidy)

RCC can have associations with VHL, smoking , acquire renal cystic disease in ESRD , ADPKD

In the upper urinary tracts (ie renal pelvis and calyces ) however , transitional cell cancer is commoner . For these the cell of origin is the DCT and the collecting ducts These are supposed to be associated with analgesic abuse , Balkan nephropathy , smoking , cyclophosphamide & lynch syndrome

The renal cell carcinomas can have para neoplastic manifestations like 1. 2. 3. 4. hypercalcemia non metastatic hepatopathy (STAUFFERS SYNDROME) acquired dysfibrinogenemia erythrocytosis (and not polycythemia . CSDT specifically says so)

in addition , Htn & anemia may also occur hyper calcemia , anemia , increased LDH ,cachexia , karnofsky score < 80 are considered to be bad prognostic indicators

A RCC invading the IVC may present with pedal edema Remember IVC invasion is more likely in a right sided lesion A left sided lesion can manifest as acute varicocele

DIAGNOSTIC MODALITIES

USG .. Its main role is to differentiate a benign simple cortical cyst from a solid mass (remember malignancy is the most common solid mass in the kidney) CT .. It is the best diagnostic moality . A CT with iv contrast typically shows a solid , enhancing mass due to the high vascularity of the tumor An oral contrast helps in the delineation of the involved retro peritoneal nodes MRI This is the best investigation to demonstrate renal vein or IVC invasion Vena cavagram is also helpful for the same X ray abdomen In this a renal mass with CENTRAL calcification is considered more likely to be malignant

IVP .. Apart from USG , this will also help in differentiating a simple cortical cyst from a RCC because the former will typically show beak sign DMSA .. to study the parenchyma DTPA .. for functional study This can therefore differentiate a malignancy from hypertrophied renal column of bertin , as the latter shows increased uptake (PSEUDO TUMOR) while the former shows decreased uptake MAG 3 .. for both structural and functional study LFT , RFT , urine cytology , cystoscopy (to detect drop metastasis especially from the transitional cell carcinomas of the renal pelvis) , bone scan are also done Remember THE MOST COMMON SITE OF METASTASIS OF RCC IS THE LUNG FOLLOWED BY THE BONE , BRAIN AND LIVER IN ADVANCED STAGES THE MOST COMMON PRIMARY IN A CASE OF THE RENAL SECONDARIES IS ALSO LUNG Staging Stage 1 the tumor is confined within the renal capsule Stage 2 .. the tumor extends into the peri renal fat or adrenal but limited within gerotas fascia Stage 3a tumor extends into the renal vein or IVC Stage 3b .. tumor extends into the draining nodes

Stage 3c .. it is stage 3a+b Stage 4a .. spread to adjacent organs (other than the adrenals) Stage 4b .. distant metastasis

TREATMENT For stages 1, 2,3a A radical nephrectomy is done This involves the resection the entire kidney , gerotas fascia , proximal half of ureter & renal hilar nodes During this procedure RENAL ARTERY IS TIE FIRST FOLLOWE BY THE RENAL VEIN Para aotic node dissection is not currently recommended

However in cases of tumor more than 4 cm , associated diabetes , recurrent UTI , ESRD a partial nephrectomy is suggested In case of a RCC with a solitary pulmonary secondary , joint resection of both may be done

For the metastatic disease , Both chemo an radio therapy are of limited value Immuno therapy (with cyto reductive nephrectomy ) using LAK cells ,IFN & IL2 was found to be useful in some cases But IL 2 can produce capillary leak syndrome Since a majority of RCC patients have VHL mutation (which acts through VEGF pathway) , bevacizumab is gaining importance as a therapy for metastatic RCC Nowadays sorafenib anb sunitinib are found to be very useful in metastatic disease. Their main side effect is diarrhea. They also produce the hand foot syndrome Temsirolimus is another useful drug

Certain other points .. Renal medullary cell carcinoma has an association with sickle cell trait !! RCC tumor grading is called the Fuhrmann grading Radical nephrectomy is ideally done laparoscopically But partial nephrectomy is ideally done by the open method!!(to prevent urine leak)