Renal Tumors PDF

p Renal Tumors Prof.Dr: Mohebat helmy Learning objectives:  Classify kidney tumours.  Classify urinary bladder tumours.  Discuss the pathogenesis of kidney and bladder carcinoma.  Discuss the morphology of renal carcinoma, Wilm’s tumour & bladder carcinoma.  Tumors of...

p Renal Tumors Prof.Dr: Mohebat helmy Learning objectives:  Classify kidney tumours.  Classify urinary bladder tumours.  Discuss the pathogenesis of kidney and bladder carcinoma.  Discuss the morphology of renal carcinoma, Wilm’s tumour & bladder carcinoma.  Tumors of the Kidney I- Benign: Epithelial: Cortical adenoma, Oncocytoma Mesodermal: 1-Renal fibroma or hamartoma. 2-Angiomyolipoma 3-Lipoma, leiomyoma, myxoma, hemangioma, and lymphangioma. II- Malignant: 1ry: * Epithelial: Renal cell carcinoma. *Mesodermal: Sarcoma, lymphomas and MM. * Wilm's tumor (nephroblastoma) 2ry: - Direct or lymphatic spread from adrenal, panc. - Blood stream metastasis I- Benign: Epithelial: Papilloma, inverted papilloma Mesodermal: leiomyoma and neurofibroma. Hemangioma either capillary or cavernous. Its rupture causes severe hematuria II- Malignant: (a) Transitional cell carcinoma (b) Squamous cell carcinoma  Renal cell carcinoma (Adenocarcinoma)  Incidence:  It represents 1-3% of all visceral cancer,  85% of renal cancer in adults.  The tumor is more in males and occurs between 40-70ys.  Origin: It arises from the epithelium of the renal tubules or from a cortical adenoma.  Risk factors:  * (the most significant) it double its incidence.  * Obesity. *Hypertension.  *Unopposed estrogen therapy  *Long term renal dialysis.  *Exposure to arsenic, asbestos, petroleum products & heavy metals.  *Increased in patient with chronic renal failure, acquired cystic disease, tubular sclerosis.  * Genetic factors Etiology of RCC  * Sporadic: Most common.  * Familial: autosomal-dominant familial cancer occurs  - Hereditary (familial) clear cell carcinoma  * Confined to kidney involving VHL gene.  * Bilateral, often multiple renal cell carcinomas of clear cell type.  - Hereditary papillary carcinoma  * Multiple, bilateral tumor  * Papillary histology. Clinical effects:  * ainless hematuria.  * ain in loin  * alpable mass.  * araneaplastic syndromes: polycythemia, hypercalcemia, hypertension, hepatic dysfunction, feminization, musculinization, Spread of the tumor: : to renal medulla, renal capsule to surrounding structures and renal vein to inferior vena cava. early to lung, bone and liver. to para aortic lymph nodes. 1-Clear RCC  Incidence & pathogenesis:  *The most common (70-80%).  *It may be familial or associated with VHL disease OR mostly sporadic 95%.  *There is loss of sequences of the short arm of chromosome 3 _ occurs by deletion or unbalanced chromosomal translocation (3,6- 3,8- 3,11), or somatic mutations which indicate that VHL gene acts as a tumor suppressor gene (in 80% of clear cell renal cell carcinoma). Gross Picture:  Site:Arise mostly from proximal tubular epithelium.Tumor arises in any part but commonly affects the poles particularly the upper pole.  Shape:Solitary, unilateral, Spherical mass.  Size: varies in size and distorts the renal outline.  Color:Bright yellow (due to lipid accumulation).  C/S: Gray- white with large areas of ischemic opaque necrosis, foci of hemorrhage & areas of softening.  The margins are sharply-defined. Microscopic Picture: Solid trabecular or tubular pattern. Round or polygonal cells with abundant clear or granular cytoplasm Have delicate branching vasculature. May be with cystic & solid areas. Mostly differentiated but may show nuclear atypia, bizarre nuclei & giant cells. 2-Papillary RCC  Incidence & pathogenesis:  *10-15%.  *Occurs in both familial and sporadic forms.  *Associated with 3p deletions.  *Common---- trisomies 7, 16, 17, loss of Y in males with sporadic forms & triosomy 7 in familial forms.  *Papillary RCC -------- 2nd gene on chromosome 1 in sporadic forms, (x,1) translocation.  Gross Picture:  *Arise from distal convoluted tubules.  *Multifocal bilateral.  *Hemorrhagic _ cystic  *As it enlarge _ bulge into calyces & pelvis _ may fungate through the walls of collecting system _ even enter the ureter.  *High tendency to invade the renal vien grow as solid column _ may extend to inf. Vena cava _ even Rt. side of the heart.   Microscopic Picture:  Cuboidal or law columnar cells arranged in papillary formation.  In the papillary core interstital foam cells.  Psammoma bodies.  Stroma scanty but highly vascular.  In the core of papillary RCC, we can identify:  Neutrophils  Foam histiocytes  Blastemal component  Malignant spindle cells  Plasma cells  Chromophobe RCC  Incidence & pathogenesis:  5% with multiple chromosomal losses.  It arises from the epithelium lining cortical part of collecting ducts  Grossly: Solid tumor with light brown cut surface.  Microscopic picture: _ Large cells with prominent cell membranes. _ Pale esinophilic cytoplasm with perinnuclear halo. _ In solid sheets With concentration of cell around blood vessels. Collecting duct (Bellini duct) carcinoma  Incidence & pathogenesis:  *1% or less.  *Number of chromosomal losses and deletions. It arises from the medullary part of collecting ducts. It has a very aggressive behavior.  Gross Picture:  *Rare variant  * It usually located in the medulla of the kidney.  Microscopic Picture:  *Irregular channels or tubules lined by highly atypical epithelial cells with hobnail pattern. G/P: Firm, white-grey tumors with ill-defined, infiltrative borders, often with multinodularity. Note the separate tumor nodules in the hilar fat M/P:Tubular (a) or tubulopapillary architecture with other admixed patterns, including papillary with true fibrovascular cores (b), solid cords and sheets (c), and cribriform (d) High-grade cytologic features in collecting duct carcinoma, including high nuclear to cytoplasmic ratio, nuclear enlargement and irregularity, pleomorphism, and prominent nucleoli Staging:TNM classification of RCC:  T1: Limited to kidney, ≤2.5cm  T2: Limited to kidney >2.5 cm  T3:T3-a Invades perinephric tissue or adrenal glands T3-b Invades renal vein or vena cava T3-c Extends to vena cava above diaphragm  T4 Invade beyond Gerota fascia.  Wilm's tumor (Nephroblastoma or Embryoma)  Definition: It is a childhood tumor derived from the renal blastema.  Incidence: The peak incidence is 2-4 ys.  The tumor constitutes 20% of malignant tumors of children and is bilateral in 5- 10% of cases.  Morphology  Gross Picture: Size:Large Shape:Rounded or lobulate,well circumscribed. Consistency: Soft. Cut section is pale gray and shows areas of cystic changes.  Microscopic picture: The tumor consists of three components:  (1) Cellular nests and sheets of primitive blastemal cells, round or oval with scanty cytoplasm.  (2) Mesenchymal component of fibrous tissue, smooth muscle, striated muscle, bone and cartilage.  (3) Epithelial component of embryonic tubules and glomeruloid structures. Solid, Solid, expansively growing growing expansively the nephrectomy specimen. encapsulated / sharply demarcated tumor occupying encapsulated / most of sharply demarcated tumor occupying most of the nephrectomy specimen. The images show the characteristic three components:  Clinical Features: The child presents with a large abdominal mass. Hematuria and pain in the abdomen occurs after trauma. Combined treatment by radiotherapy, chemotherapy and surgery markedly reduces the recurrence rate.  Spread:  (1) Local infiltration of the kidney capsule and surrounding structures.  (2) Blood spread early to the lung, liver, bone, and brain.  (3) Lymphatic spread to the para-aortic lymph nodes.  Staging of pediatric renal cell tumor:  Stage I: Limited to kidney and completely resected  Stage II: Tumor infiltrates beyond kidney, but completely resected  Stage III: Residual non hematogenous tumor confined to abdomen  Stage IV: Hematogenous metastasis  Stage V: Bilateral renal involvement at diagnosis  Transitional Cell Carcinoma of the Renal Pelvis  These are small tumors that can block the urinary outflow and lead to palpable hydronephrosis and loin pain. On histologic examination they are the exact counterpart of those found in urinary bladder. In 50% of cases there is a preexisting or concomitant bladder urothelial tumor.  Tumors of the ureter  Primary neoplasia is very rare but metastatic spread is common. They include:  Benign: of mesenchymal origin (fibrous tissue, muscle, Blood vessels & lymphatic) e.g. fibroepithelial polyp, leiomyoma.  Malignant:  Primary: of urothelial origin (transitional cell carcinoma).  Secondaries