!! 2 to 3% of All New Visceral Cancers.!! Peak Incidence is 6th Decade!! M:F = 2:1

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1 !! Kathleen M. O Toole, M.D.!! 2 to 3% of All New Visceral Cancers!! Peak Incidence is 6th Decade!! M:F = 2:1!! Grossly is a Bright Yellow, Necrotic Mass with a Pseudocapsule 1

2 !!Conventional RCC! Clear cell, granular cell, spindle cell!!papillary RCC!!Chromophobe cell RCC! Polygonal cell, eosinophilic cell!!collecting Duct Carcinoma 2

3 !!Classic Triad = (19%) = Hematuria, Abdominal Mass and CVA Pain!!Paraneoplatic Syndromes = 25%!Hypertension, Cushing s Syndrome, Polycythemia, Hypercalcemia!!von Hippel-Lindau Disease!!Amplification of c-myc Expression!!CRC: 3p-, other losses/gains!!papillary: 7+, 17+, 3+, Y-, t(x;1), t(x;17)!!chromophobe: Y-,1-, 2-, 6-,10-, 13-,17-,21-!!Collecting Duct: 1-, 6-, 14-,15-, 22-!! Typically, blood-borne metastasis Lungs Brain Bone Unusual sites!! Lymphatic metastasis Regional nodes Para-aortic nodes 3

4 !! 85% of Malignant Pediatric Renal Neoplasms!! Age 2-5 Years!! 5% Bilateral; 5% Multicentric!! Large, Circumscribed and Lobulated Mass!! Triphasic Histology, with Blastemic, Epithelial and Stromal Elements!! Prognosis Depends on Stage, and Presence of Anaplasia 4

5 !!Sporadic Aniridia!!Hypogenitalism!!Mental Retardation!!Urogenital Tract Anomalies!!Deletion of Chromosome 11p13!!Beckwith-Wiedmann Syndrome!!Drash Syndrome!!TNM System - T (extent of local spread) - N (presence or absence of regional node mets) - M (presence or absence of distant metastasis)!! Strict Diagnostic Criteria Must be Used!! Grossly Consists of a Well Circumscribed, Mahogany-Brown, Bosselated Mass, with a Central Stellate Scar!! Histologically Comprised of Nests, Trabecular and Tubular Structures Made Up of Large, Polygonal Cells with Eosinophilic Granular Cytoplasm!! Clear Cell Differentiation, Necrosis, or Papillary Architecture Rule Out Diagnosis 5

6 ! 50% Are Associated With Tuberous Sclerosis! Grossly Single to Multiple Bright Yellow Neoplasms, Which Vary in Size! Histologically Comprised of Large, Thick-Walled Blood Vessels, Mature Smooth Muscle and Adipose Tissue! Controversy- Neoplasm vs Hamartoma!!Soft tissue tumors! Benign, malignant!!metastatic carcinoma!!malignant Lymphoma 6

7 !! Benign- condyloma!! HPV associated, serotypes 6 and 11!! Malignant- squamous cell carcinoma!! Uncircumcised penises!! High incidence in South America, Africa, Asia (not Japan)!! Often HPV associated, serotypes 16 and 18!! 7

8 !!Urinary Retention Symptoms!! Hesitancy, frequency, nocturia, incomplete emptying!! Due to compression of urethra by enlarged prostate!! Most of the enlargement is periurethral Unknown? Disturbance of androgen-estrogen ratio? Stromal induction!! Smooth or nodular!! Firm, elastic or rubbery consistency!! Cut surface is moist, and milky fluid is usually seen!! Cystic and honey-combed areas are usual!! Marked nodularity 8

9 ! Greatly increased number of acini! Increased size of acini! Saw-toothed appearance of acini! Preservation of double cell layer inner - epithelium, usually columnar outer - basal cells! Increased amount of fibromuscular stroma Most of the hyperplasia occurs in the periurethral portion of the gland. 9

10 !!Unknown!!Somehow related to androgens!!genetic Factors! Racial differences! Familial carcinomas!!environmental factors!!most cases in US detected following workup of abnormal PSA!!Rarely, urinary tract symptoms!!bone pain in advanced cases! Gland may be normal, large, or small! Most cancers occur peripherally, in the subcapsular region! Cancer is usually gray-white, hard, and gritty. Loss of nodularity.! Ill-defined borders.!!> 95% are adenocarcinomas Mostofi I III nuclear features Gleason pattern 10

11 ! T= local extent T2-organ confined T3-extracapsular or seminal vesical invasion T4-invasion of bladder or rectum! N=regional nodes! M=distant metastsis 11

12 !!Prostatic Intraepithelial Neoplasia!!Architecturally benign acini or ducts lined by cytologically atypical cells!!precursor Lesion for some? Most cancers!!repeat biopsy recommended 12

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