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1. Presenter : Dr.Ibsa Daba ( Urology resident) Moderator: Dr.Messay ( Consultant Urologist ) Renal tumors: Epidemiology, Pathology ,symptoms and diagnosis ,staging

2. Out line Background Classification Renal cysts Benign renal tumors Malignant renal tumors Stage and grade of RCC Diagnosis 2

3. Introduction Renal masses can be classified by Barbaric (1994 ),based on pathologic features M alignant, B enign , or I nflammatory 3 RENAL MASSES CLASSIFIED BY PATHOLOGIC FEATURES MALIGNANT BENIGN INFLAMMATORY RENAL CELL CARCINOMA UROTHELIAL BASED SARCOMA WILMS TUMOR CARCINIOD LYMPHOMA LEUKEMIA METASTASIS SIMPLE CYST ANGIOMYOLIPOMA ONCOYTOMA METANEPHRIC ADENOMA CYSTIC NEPHROMA LEIOMYOMA FIBROMA HEMANGIOMA ABSCESS FOCAL PYELONEPHRITIS XANTHOGRANULOMATOU NEPHRITIS INFECTED ENAL CYST TUBERCULOSIS RHEUMATIC GRANULOMA

4. CLASSIFICATION Classified based on radiographic appearance Simple cystic , Complex cystic Solid. 4

5. Simple Cortical Renal cysts Older population Incidental dx 50 % in older than 50 years of age Asymptomatic Calcification in less than 1% Doesn’t enhance but distort adjacent parenchyma to produce beak or claw sign Management is conservative 5

6. Complex renal cysts B enign Renal cyst The most common benign renal lesions Risk factors of increasing age male gender hypertension worsening renal function Presentation Usually asymptomatic, pain from local expansion Palpable mass, hematuria in ADPKD Rarely come with pain and hypertension . Sporadic , Acquired , or Genetic in their origin . ADPKD or ARPKD Acquired renal cystic disease can be associated with RCC is 7% 6

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8. T reatmen t Bosniak class 1, 2: Benign no follow-up or treatment Bosniak class 2F ( for follow up): Imaging follow-up ○ CT/MR, interval time: 6, 12 months ○ Concerning imaging features at follow-up: Enhancing solid nodules or T hick septations I ncreased thickness of enhancing wall Cyst growth is not correlated with progression to malignancy Recommended length of follow-up: 4-5 years 8 Bosniak Class 3, 4: Treatment Surgical resection Partial nephrectomy or ablation biopsy is controversial ○ For local symptoms Aspiration Cyst decortication Cyst resection Sclerotherapy Arterial embolization, Nephrectomy

9. Oncocytoma Epidemiology O ncocytoma is the most common benign enhancing renal mass. Male and common 7 th decade Associated with Birt -Hogg- Dube syndrome: characterized by pulmonary cysts, spontaneous pneumothoraces , and fibrofolliculomas 25% of renal masses less than 3cm represent oncoytoma Reports of metastatic disease (extremely rare) are likely cases of eosinophilic chromophobe RCC . 9

10. Pathology D erived from the distal renal tubules homogeneous , and well circumscribed with a pseudocapsule and t ypically a central stellate scar Perirenal fat invasion and renal vein invasion. Differentiate from chromophobe R CC immunohistochemistry(cytokeratin 7) 10

11. Presentation Asymptomatic On CT, is well defined with a relatively sharp interface with the normal renal parenchyma The central stellate scar is best seen with CT A " spoke wheel Pattern of vessels penetrating into the center of the tumor is felt to be characteristic Renal biopsy accompanied by immunostain 11

12. ANGIOMYOLIPOMA Composed of dysmorphic blood vessels, smooth muscle, adipose tissue Sporadically or gene tical syndromes TSC ( 55 90%)and lymphangioleimyomatosis . Common in Women and peak in 4th and 5th decade 12

13. Pathology P ossibly derived from perivascular epithelioid cell tumors ( PECOMAs ). T umors are well circumscribed with a tan, pink , or yellow surface, Composed of blood vessels ,spindle cells around vessels, adipocytes M etastatic disease -one-third of reported cases Strongly expresses estrogen receptor- β ,progesterone receptor and androgen receptor, which explain postpubertal female predominance 13

14. Presentation and diagnosis M acroscopic fat on CT or MRI is diagnostic of AML . Intralesional fat (−15 to − 20 [ HU]) on non enhanced series is diagnostic CT-angiography shows aneurysmal dilation in 50% of angiomyolipomas Fat poor AML(4-14%) MRI indicated USG- less reliable a well-circumscribed, highly echogenic lesion with shadowing . 14 Asymptomatic Symptoms are more likely in larger AML >4cm Flank pain, retroperitoneal hemorrhage

15. Nonenhanced CT shows a mass (arrows) with fatty attenuation in the upper pole of right kidney Contrast-enhanced coronal CT shows mottled and linear structures (arrows) with mild enhancement in the mass. echogenic masses ( arrows ). the same or higher than that of renal sinus. The masses do not have peritumoral halo or intratumoral cysts,

16. OTHER BENIGN RENAL TUMORS Adenoma of the kidneys(Renal cortical adenoma) Cystic Nephroma Leiomyoma Metanephric adenoma Mixed Epithelial/ Stromal Tumor F ibromas L ipomas L ymphangiomas Hemangiomas R eninoma 16

17. EPIDEMIOLOGY , AETIOLOGY AND PATHOLOGY RENAL CELL CARCINOMA 17

18. Epidemiology 3% of all cancers Last two decades 99,200 new cases and 39,200 mortality related within European union . 90% of kidney malignancies Predominance in men over women with 15:1 Higher incidence In older 18

19. Etiology S moking , O besity , and Hypertension and Diabetes Exposure to asbestos, heavy metals , petroleum products Regular use of NSAIDs First degree with RCC Retroperitoneal radiation for testicular tumors and 19

20. Familial RCC 20

21. Pathology There are three main RCC types: C lear cell ( ccRCC )- 70-80% Papillary ( pRCC type I and II) and 10-15% C hromophobe ( chRCC - 3 -5% H istological diagnosis includes besides RCC type Nuclear G rade Sarcomatoid features Vascular invasion Tumour necrosis I nvasion of collecting system Four-tiered WHO/ISUP (International Society of Urological Pathology ) 21

22. Cont…. O void and circumscribed by a pseudocapsule Aggressive local behavior not uncommon, can be expressed . Invasion of renal capsule ,sinus, collecting system found in 20 % Spread to adjacent organs abdominal wall is often preclude by Gerota fascia , Predilection of venous system and inlvolved in 10% (renal vein and IVC and ascend up to right atrium) Most sporadic RCC are solitary Bilateral involvement of 2 to 4 % in sporadic RCC Muliticenter ore common in familial especially VHL All RCCs are, by definition, adenocarcinomas 22

23. Clear-cell RCC Most common type 70 to 80 % 95 % sporadic and 5 % familial VHL mutation Worse prognosis compared to pRCC and chRCC von Hippel-Lindau disease 30 to 50% RCC Third to 5 th decade bilateral and multifocal involvement 23

24. 24 well circumscribed and a capsule is usually absent The cut surface is golden-yellow, often with hemorrhage and necrosis highly vascular, containing a network of delicate vascular sinusoids On microscopic examination, include clear cells, granular or eosinophilic cells, or mixed types. 3-5% percent RCCs demonstrate sarcomatoid features, clear cell RCC is more likely to exhibit venous tumor extension ( exception

25. Papillary Renal Cell Carcinoma Papillary RCC is the second(10%–15%) most commonly encountered morph type Type 1 -Trisomy of chromosome 7 and 17 Altered MET proto oncogene status, present in 81% of sporadic cases multicentricity , which approaches 40% in patients with ESRD Type 1-carries a better prognosis than clear cell RCC, Type 2 -is similar or worse than clear cell RCC 25

26. Proximal tubule On microscopic examination, most tumors consist of basophilic or eosinophilic cells arranged in papillary or tubular configuration

27. Chromophobe RCC Represents 3% to 5% of all RCCs D erived from the distal convoluted tubules BHD syndrome, but most cases are sporadic Loss of chromosomes Y, 1, 2, 6, 10, 13, 17 and 21 are typical genetic changes . The prognosis is relatively good, with high 5-year recurrence-free survival (RFS), and 10-year CSS 27 .

28. pale tan, relatively homogenous and tough, well-demarcated mass without a capsul The tumor cells typically exhibit a relatively transparent cytoplasm with a fine reticular pattern that has been described as a “plant cell” appearance

29. Other renal tumors Other renal tumours constitute the remaining renal cortical tumours . These include a variety of uncommon, sporadic, and familial carcinomas, some only recently described, as well as a group of unclassified carcinomas 29

30. Diagnosis Sign and symptom > 50% of RCC are detected incidentally Symptoms associated with RCC can be due to: L ocal tumor growth Metastatic disease P araneoplastic syndrome Hemorrhage 30

31. Paraneoplastic syndromes A pproximately 30% of patients with symptomatic RCCs Over production of 1,25-dihydroxycholecalciferol , renin, erythropoietin, and various prostaglandins 31

32. L aboratory Hemoglobin ,ESR Serum creatinine , CrCl Liver function tests: Stauffer syndrome alkaline phosphatase(liver metastasis , bone met) lactate dehydrogenase (LDH) S erum calcium- Paraneoplastic syndromes U rinary cytology –upper tract TCC , collecting duct Ca Split renal function , Renal scintigraphy 32

33. Ultrasound In distinguishing solid from cystic renal masses. A renal adenocarcinoma is usually seen as a solid mass, cystic regions areas of hemorrhage or necrosis are common . Intra op Imaging- for intra renal and nephron sparing USG failed to detect lesion less than1 cm and cannot stage 33

34. Staging and Imaging description Renal invasion( exophytic or intrarenal ) Involvement of collecting system IVC invasion Tumor vascularity Local or distant metastasis Perinephric invasion Lymph node enlargement Adrenal metastasis 34

35. Staging, AJCC TNM classification in 2016 35

36. Renal protocol imaging Non contrast examination of liver and kidneys Post contrast during nephrogenic phase Excretory phase The echogenicity of renal carcinomas is variable. Clear cell renal carcinomas tend to be heterogeneous , Hypervascular masses, often with central areas of necrosis 36 CT scan

37. RCC staging Tumor less than 4cm Confined to renal renal capsule Best prognosis Partial nephrectomy Tumor measuring 4-7cm Confined to renal capsule 37 Stage Tia Stage Tib

38. Stage T2 Tumour > 7 cm in greatest dimension , Limited to the kidney In this image Mass protruding to pelvis Lower pole renal mass 38

39. Stage T3 Perinephric fat stranding IVC and renal venous tumor thrombus and IVC involvement are 78% and 96%, respectively CT findings suggestive of venous involvement include venous enlargement, abrupt change in the caliber of the vein filling defects, 39

40. Stage T4 Tumor extends to adjacent structures and ipsilateral adrenal gland. 40

41. Other findings on CT san Perinephric hemorrhage either a RCC or an angiomyolipoma Tumors with lymphadenopathy 41

42. MRI Metastatic evaluation Locally advanced malignant disease Equivocal venous involvement Allergy to IV contrast Staging of IVC throbus Chest CT for patients with pulmonary symptoms Vertebrae ,imaged ribs,pelvic bones( expansile lytic lesions) Liver function tests Bone scintiscan 42

43. Anatomic classification systems ( R.E.N.A.L . nephrometry score) R.E.N.A.L. nephrometry score , Using the scoring system, tumor complexity is determined: Low complexity - N ephrometry score = 4–6 M oderate complexity - N ephrometry score = 7–9 H igh complexity - N ephrometry score = 10–12 43

44. Summary 44

45. references RENAL Nephrometry Scoring System : The Radiologist’s Perspective, AJR 2012; 199:W355–W359 45