Renal Tumors PDF

Renal Tumors  BENIGN RENAL TUMORS:  The most common benign tumor: Angiomyolipoma, oncocytoma  Angiomyolipoma: consists of a BVs (Angio) & SMCs (myo) & fat (lipo), seen in 25-50% of patients with tuberous sclerosis (TS)  TS: disease in which there is loss of function mutations in...

Renal Tumors  BENIGN RENAL TUMORS:  The most common benign tumor: Angiomyolipoma, oncocytoma  Angiomyolipoma: consists of a BVs (Angio) & SMCs (myo) & fat (lipo), seen in 25-50% of patients with tuberous sclerosis (TS)  TS: disease in which there is loss of function mutations in TS1 + TS2 gene  these patients have Brain Tumors and skin lesions (Ash-Leaf Pathches)  Oncocytoma:  10% of renal tumors & it's encapsulated tan or brown in color (mahogany)  Eosinophilic cells packed with Mitochondria  genetic changes: loss of chromosomes 1 and Y  RENAL CELL CARCINOMA (RCC) (Malignant)  1-3% of all visceral cancers, 85% of all renal cancer  Most common in elderly (60-70 years), M: F = 2:1  Risk factors:  Smoking, obesity, hypertension  Unopposed estrogen Rx (Tamoxifen)  Asbestos, petroleum products & heavy metals  CRF & acquired cystic disease (30 folds, not considered as premalignant)  Familial: 4% (most common are sporadic)  Von Hippel-Lindau (VHL) sy: mutations in VHL gene-ch 3  Hereditary clear cell carcinoma  Hereditary papillary carcinoma  Morphology of RCC: Grossly  Mainly polar, spherical yellow (fill with fat) Variegated tumor  with hemorrhagic, necrotic and cystic areas.  May extend into renal vein (metastasis)  Morphology of RCC: Microscopically  Clear cell carcinoma  Most common ,70-80% of renal CA  Clear cells with clear or granular Cytoplasm  Majority are sporadic but there are Familial forms like vHL  Papillary RCC  10-15% of all renal CA, Papillary growth pattern  Frequently multifocal and bilateral, Appear as early stage tumor  Sporadic: Trisomy 7,16,17 loss of Y Chromosome  Familial: MET protooncogene on Chromosome 7and trisomy 7 Page | 0   Chromophobe RCC  5% of all RCC, Arise from cortical collecting ducts or Their intercalated cells  Multiple losses of entire chromosomes (1,2,6,10,13,17,21)  General good prognosis (have the same origin) as oncocytoma which is benign Tumor  Usually no mets (only in 10% of cases) & no LN involvement, No lymphovascular invasion  Microscope: resinoid nucleus, halo around the nucleus and a distinct cell membrane  Sarcomatoid carcinoma: dedifferentiation towards sarcoma, but its carcinoma in origin  Clinical features of RCC  Most cases are Asymptomatic/incidental finding  Hematuria (50%), costovertebral pain, mass (all 10%)  Constitutional symptoms (Fever /malaise/ weight loss)  Advanced stage present with mets (lungs & bones)  Paraneoplastic syndromes:  Polycythemia 5-10% (EPO), Hypercalcemia (PTHrp)  Cushing’s syndrome (ACTH), HTN (Renin)  Feminization or masculinization  Prognosis: 5 years’ survival is around 70%  Stage is the most important prognostic factor, depends on:  Tumor size, R.V involvement, extending outside the capsule of the kidney  If the tumor is small (2-3cm) usually undergo partial nephrectomy (not radical)  NEPHROBLASTOMA (WILM’STUMOR)  Uncommon tumor but one of the Commonest childhood neoplasms (up to 5 years old)  Risk factors:  Congenital malformations with abnormalities of chromosome 11 (WT-1 & WT-2) -stand for wilms tumor suppressor gene  These patients usually have denys drash syndrome or beckwith wiedemann syndrome  Grossly: Large well circumscribed soft Tan-gray homogenous Tumor  Microscopically: Blastemal, stromal and epithelial elements  Presents with abdominal mass (most common), hematuria, Pain or HTN  Prognosis: currently 90% long term survival  Renal pelvis carcinoma  The lining of the renal pelvis is transitional epithelium (urethelium)  Types: transitional CC or squamous CC (with long standing irritation)  5-10% of renal neoplasms, Often small and present early with:  Painless Hematuria  Pain or mass due to hydronephrosis (due to obstruction)  May be multifocal  Prognosis: Variable, depend on stage and grade, Despite removal by nephrectomy, the 5y survival rate is 50% (bad 😔) Page | 1  Ureters  Like the renal pelvis, the lining of the ureters is transitional epithelium (urethelium)  types: transitional CC or squamous CC  Sites of narrowing: Ureteropelvic & Ureterovesical junction, Crossing of iliac vessels.  In female pelvis, the ureters lie close to uterine Arteries and the cervix.  Operations of the Female genital tract & diseases of the Cervix & uterus may affect ureters  Causes of Ureteral Obstruction:  Intrinsic: Calculi /Strictures /Tumors /Blood clots /Neurogenic  Extrinsic: Pregnancy/Periureteral inflammation/Endometriosis /Tumors  Endometriosis: Presence of the endometrial gland & stromal cells outside endometrium  Usually in fallopian tubes & ovary & abdominal wall especially after the cesarean section  Cystitis: Inflammation of the urinary bladder  Bacterial cystitis:  Etiology:  Ecoli (most common)  Proteus vulgaris  Klebsiella pneumonia  Pseudomonas aeruginosa  Streptococcus fecalis  Chlamydia  ureaplasma  Mycoplasma  Chlamydia, ureaplasma & mycoplasma have a tendency to develop stones.  Predisposing factors:  Sexually active females  Diverticula  Short female urethra  Benign prostatic hyperplasia (BPH)  Foreign bodies (stones, catheter)  Diagnosis: depends on symptoms & culture  Symptoms: inflammation, frequency of urine, hematuria  Urine analysis & culture  Biopsy: acute inflammation with erosions of the urinary bladder  Schistosomal Cystitis:  Etiology: Scistosoma hematobium (parasitic infection)  Pathogenesis:  Cercariae penetrates the skin → Mature worms lay eggs in the bladder wall  Eggs irrigate the bladder causing granulomatous inflammation → calcifying the eggs  Complications: lower UTI, hydroureter, Hydronephrosis  Long standing schistosomal cystitis ↑ risk of developing Bladder carcinoma (mostly SCC) Page | 2  Bladder cancer  The lining of the urinary bladder is transitional epithelium (urethelium)  7% of male CA, 2% of female CA, Mean age 65Y (50-80) (M: F 3:1)  >90% are transitional (urothelial) cell Carcinoma.  Etiology: mostly unknown, but there are some possible causes:  Induction time ~22Y  Cigarette smoking, Cyclophosphamide  Phenacetin containing Analgesics, S. Hematobium Infestation  Dye & rubber Industry:  Aromatic amines 2 –naphthylamine  4-aminobiphenyl, Benzidine, Azo dyes  Could be benign or malignant  Papilloma: only benign tumor in the urothelial neoplasms  Not very common, usually seen in children  Very low tendency to transform into carcinoma (1-2%)  Low grade TCC & High grade TCC  Papilloma & NIP carcinoma: same pattern (differ in Histomorphologic F).  Most of TCCs express papillary pattern  IPC: invade the lamina Propria & SMCs  Flat non-invasive carcinoma: does not invade, Like CIS.  Flat invasive carcinoma: invades to underlying Structures  Urothelial carcinoma in situ:  High grade intraurothelial neoplasia  A non-papillary, flat lesion (Surface epithelium contains cells that are Cytologically malignant).  Accounts for less than 1-3% of urothelial Neoplasms  Is seen in association with invasive Carcinoma (45-65%)  Asymptomatic or with frequency, urgency or even hematuria  Other bladder tumors: SCC 5% (schistosomiasis & tobacco smoking), Adenocarcinoma 2%, Sarcomas, Paraganglioma, Carcinoid Page | 3  Miscellaneous  Summary of genetic relationship of RCC: In papillary RCC: Macrophages in the papillae  To measure proteinuria:  Qualitative (urine dipstick): +1 → +4  Quantitative: protein in 24 hours  Types of ectopic kidneys:  Simple: proper side but abnormal location  Crossed: opposite side  NOTES:  Biopsy of the kidney shouldn’t be taken directly (risk of mets)  PT & PTT Should be checked before operations  Maignant HTN diagnosed clinically  IF in amyloidosis: monoclonal staining of accumulated amyloid Page | 4