RENAL CELL CARCINOMA 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 Pseud

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1 GENITOURINARY PATHOLOGY Kathleen M. O Toole Toole, M.D.

2 RENAL CELL CARCINOMA 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

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7 HISTOLOGIC SUBTYPES Conventional RCC Clear cell, granular cell, spindle cell Papillary RCC, Type 1 or Type 2 Chromophobe cell RCC Polygonal cell, eosinophilic cell Collecting Duct Carcinoma Translocation Carcinoma Miscellaneous rare entities

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18 RCC ASSOCIATIONS Classic Triad = (19%) = Hematuria, Abdominal Mass and CVA Pain Paraneoplastic Syndromes = 25% Hypertension, Cushing s s Syndrome, Polycythemia, Hypercalcemia von Hippel-Lindau Disease Amplification of c-myc Expression

19 MOLECULAR GENETICS CRC: 3p-, other losses/gains Papillary: 7+, 17+, 3+, Y-, t(x;1), t(x;17) Chromophobe: Y-,1-,, 2-, 6-,10-,, 13-,17,17-,21,21- Collecting duct: 1q32-,6p,6p-,, 8p-, 13q-, 21q-, Y- Translocation: Xp11.2 (TFE3 transcription factor)

20 METASTASES FROM CARCINOMA OF THE KIDNEY Typically, blood-borne borne metastasis Lungs Brain Bone Unusual sites Lymphatic metastasis Regional nodes Para-aortic aortic nodes

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26 STAGING (TNM) pt1: Confined to kidney, 7.0cm or less pt2: Confined to kidney, more than 7.0cm pt3: Invades into major veins (VC or branches), perinephric or renal sinus fat pt4: Invades through Gerota s fascia, including direct invasion of adrenal pn: Involvement of regional nodes pm: Distant metastasis

27 NEPHROBLASTOMA (WILMS TUMOR) 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

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32 WILMS TUMOR ASSOCIATIONS Sporadic Aniridia Hypogenitalism Mental Retardation Urogenital Tract Anomalies Deletion of Chromosome 11p13 Beckwith-Wiedemann Syndrome Drash Syndrome

33 WILMS STAGING I Tumor limited it to kidney and resected completely l II III IV Tumor extends beyond kidney and resected completely Tumor penetrates renal capsule Infiltration ti into renal sinus vessels Infiltration to adjacent organs or vena cava, but completely resected Biopsy of tumor before removal Tumor spillage focally Residual tumor present and confined to the abdomen Resection margins positive for tumor Transected tumor thrombus Inoperable tumor Lymph nodes of abdomen or pelvis involved by tumor Tumor implants on peritoneal surfaces Tumor spillage into peritoneal cavity Hematogenous or lymph node metastasis outside abdomen and pelvis V Bilateral renal involvement at diagnosis Tumor in each kidney should be substaged separately

34 ONCOCYTOMA 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

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37 ANGIOMYOLIPOMA 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

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41 MISCELLANEOUS RENAL MASSES Soft tissue tumors Benign, malignant Metastatic carcinoma Malignant Lymphoma

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46 PENILE NEOPLASMS 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

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53 BENIGN PROSTATIC HYPERTROPHY Urinary Retention Symptoms Hesitancy, frequency, nocturia, incomplete emptying Due to compression of urethra by enlarged prostate Most of the enlargement is periurethral

54 ETIOLOGY Unknown? Disturbance of androgen-estrogen estrogen ratio? Stromal induction

55 GROSS 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

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57 MICROSCOPIC 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

58 Most of the hyperplasia occurs in the periurethral portion of the gland.

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61 PIN Prostatic Intraepithelial Neoplasia Architecturally benign acini or ducts lined by cytologically atypical cells Precursor Lesion for some? Most cancers Repeat biopsy recommended

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63 High-grade grade PIN

64 ETIOLOGY Unknown Somehow related to androgens Genetic Factors Racial differences Familial carcinomas Environmental factors

65 PRESENTATION Most cases in US detected following work- up of abnormal PSA Rarely, urinary tract symptoms Bone pain in advanced cases

66 GROSS 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.

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69 MICROSCOPIC > 95% are adenocarcinomas

70 GRADING Mostofi I III nuclear features Gleason pattern

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80 TNM STAGING T= = local extent T2-organ confined T3-extracapsular, bladder neck, or seminal vesical invasion T4-invasion of rectum, levator, pelvic wall N=regional nodes M=distant metastsis

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