SEMESTER VI 3 RD YEAR PATHOLOGY KIDNEY TUMORS

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1 SEMESTER VI 3 RD YEAR PATHOLOGY KIDNEY TUMORS LEARNING OBJECTIVES At the end of the lecture, students should be able to: Know the pathology of renal tumors. RENAL TUMORS RENAL PAPILLARY ADENOMA Common Renal tubular epith. Cortex Pale, discrete, circumscribed Branching, papillomatous, glands, sheets Cuboidal to polygonal cells Central nuclei, scant cytopl. Histopathology similar to renal Cell Carcinoma. All tumors considered malignant until proved otherwise. RENAL FIBROMA OR HAMARTOMA Renomedullary interstitial cell tumor Small, gray-white,firm Pyramids Fibroblast- like cells and collagenous tissue Renal interstitial cell like ANGIOMYOLIPOMA

2 Benign Vessels, smooth muscle and fat Tuberous sclerosis cerebral and skin lesions --epilepsy and retardation ONCOCYTOMA Benign solitary Intercalated cells of CD Large, eosinophilic cells with small,round nuclei and large nucleoli 5-10 % of renal tumors Tan to mahogany brown, homogenous, capsulated Oncocytoma (DCT epithelia, benign) RENAL CELL CARCINOMA Familial variants Von Hippel-Lindau syndrome o --Hemangioblastoma cerebellum,retina o --Bilateral renal cysts, o --Clear cell type RCC common o Hereditery (familial) clear cell Ca. o --kidney without other manifestations of VHL gene o --same or related gene o Hereditary papillary carcinoma o --AD o --multiple, bilateral o --MET protooncogene Renal Cell Carcinoma Pathogenesis 3 major cytogenetic, genetic and histologic studies

3 Clear cell carcinoma % --cells with clear or granular cytoplasm --non papillary --sporadic (95%), familial, VHL assoc. --chromosome 3 (VHL gene) --tumor suppressor gene --HIF 1, stimulates cell growth and angiogenesis 2. Papillary carcinoma % --papillary growth pattern --familial and sporadic --trisomies 7,16, 17 and loss of Y --MET gene.(familial) Hepatocyte growth factor --PRCC.chm.1 (sporadic) 3. Chromophobe renal carcinoma --5% --multiple chm. Losses --like oncocytoma --excellent prognosis 4.Collecting duct (Bellini duct) Ca. --1% --CD in medulla --multiple chm. Losses and deletions

4 --nests of malignant cells in fibrotic stroma Morphology Gross --any portion (upper poles) PT epith. --solitary, unilateral, spherical, capsulated --bright yellow gray- white (lipid) --necrosis,he --bulge, fungate, extends to ureters --invade RENAL VEIN IVC.rt. heart Renal Cell Carcinoma:, conventional type Papillary tumors: Gross multifocal, bilateral DCT --hge. and cystic dialysis assoc. cystic disease Microscopy Clear cell Ca. --solid, trabecular, tubular --round,polygonal cells ; abundant, clear or granular cytoplasm (glycogen or lipid) Well differentiated (s.t. atypia) Papillary Ca. --cuboidal or low coloumnar cells --interstitial foam cells in pap. Core,scant stroma psammoma bodies Chromophobe renal Ca. --pale, eosinophilic cells, peri nuclear halo sheets Collecting Duct Ca --rare --irregular channels lined by atypical cells with hob-nail pattern Sarcomatoid changes

5 Clinical course Classical triad (hematuria, flank pain, mass) (<10%) Hematuria (50%) most common symptom Silent.till late constitutional symptoms Paraneoplastic syndromes (polycythemia, hypercalcemia, HTN, cushing, amyloidosis, eosinophilia, Lk. reaction) Metastases.. before local signs hematogenous (lungs) and local abdominal LN 5 year survival = 40% Rx Nephrectomy Renal Cell Carcinoma: Wilms tumor (Nephroblastoma) Wilms Tumor Nephrogenic rests: Pre malignant lesions 40% of unilateral tumors and 100% of bilateral Hyperplastic to sclerotic Increased risk in contralateral kidney also Course Chemotherapy + surgery = 5 years = 90% Children < 2 years better prognosis Increased risk of sec. tumors Overall prognosis: Good

6 Nephroblastoma (Wilm s Tumor) Most common tumor in children under 10 years of age (most between 2 and 5 years Transitional Cell Carcinoma 5-10% of adult renal ca. Urothelium of renal pelvis Benign to Invasive Ca. Etiology: Analgesic abuse, dye, rubber etc.. Multiple (pelvis, ureter, bladder) Malignant cells in urine Hematuria ; pain, hydronephrosis Prognosis.not good (Deceptively benign app.) 90% of bladder cancers Men, yrs Cigarettes, beta-nephthylamine, S.hematobium, cyclophosphsmide Multifocal Gross: Papillary (90%) invasive or non-invasive, nodular, mixed 3 grades on anaplasia and invasion

7 ANAPLASIA: increased cellularity, crowding, loss of polarity and orientation, pleomorphism, mitosis INVASION: BM of mucosa Transitional Cell Carcinoma Grade I: Transitional, increased layers, mild hyperhromasia Grade II: inc. layering, more pleomorphism, loss of polarity, mitosis. Grade III: anaplastic or undifferentiated. Marked features, cells may not be recognizable as Tr. Epith. Clinical course: Painless hematuria or urgency Recurrences of higher grade Local invasion LN metastasis Hematogenous metastasis Prognosis depends on grade