1. RENAL PARENCHYMAL NEOPLASMS

4. Benign Neoplasms of Kidney
Renal Epithelial Neoplasms
Renal Oncocytoma
Renal Adenoma
Renal Cysts
Metanephric Tumors
Mixed Tumors
Cystic Nephroma
Mesenchymal Tumors
Angiomyolipoma
Leiomyoma
Hemangioma
Fibroma
Lipoma
Neurofibroma
Rhabdomyoma

5. RENAL ONCOCYTOMA
The main clinical importance of this lesion is the difficulty in pre-operatively distinguishing it from renal cell carcinoma.
Usually solitary and unilateral
Renal oncocytomas account for approximately 5% of resected primary adult epithelial renal neoplasms.
Generally asymptomatic.

8. Radiology
The imaging appearance of oncocytomas is difficult to distinguish from RCC, they are usually resected.
The only reliable feature is evidence of metastasis or aggressive infiltration into adjacent structures, in which case the diagnosis of renal cell carcinoma can be safely made. 
Diagnosis: Pathologic

10. Treatment
Nefrectomy

11. RENAL CYSTS Fluid collection in or on the kidney.
Most common benign renal tumors.
Generally diagnosed as incidental finding of CT or USG.
Mostly unilateral and at the inferior pole.

13. Clinical Presentation
Mostly asymptomatic.
But if symptomatic:
Abdominal mass, pain
Hypertension secondary to segmental ischemia
Hematuria when ruptured into pelvicalyceal system
Diagnosis: CT, MRI, USG

14. Bosniak Classification
Classifies the cyst according to their malignant risk
Developed on CT but applied to other modalities: USG, MRI

15. 6 months

18. Signs Indicating Malignancy
Thick, irregular walls-nodularity
Thickened or scattered septae
Enhancement with IV contrast
Multilocular structure, calcifications

19. Treatment
A symptomatic renal cyst can be aspirated, but cysts have a high rate of recurrence. Percutaneous alcohol ablation has been practiced.
In a paediatric patient with normal renal function, no follow up is necessary for an incidentally- discovered renal cyst.

20. Partial/total

21. RENAL ANGIOMYOLİPOMA Rare
Can be assoc. with Tuberous Sclerosis(45-80%) (Familial inherited disorder)
Bilateral and asymptomatic
Adenoma sabaceum, mental retardation, epilepsy
Without TS: unilateral and larger
25% of the cases present with: spont. rupture and subsequent hemorrhage into retroperitoneum.

22. Typically round to oval
No capsule
Typically round to oval
Elevates the renal capsule producing a bulging smooth or irregular mass
3 major histologic components:
Mature fat cells
Immature smooth muscle cells
Dysplastic blood vessels

23. Diagnosis: CT, USG

24. Treatment <4 cm: follow up every year with CT or USG
Asymptomatic/mildly symptomatic, >4 cm: USG every 6 months
>4 cm, moderate/severe symptoms:
Renal sparing surgery
Renal arterial embolization
25-30% of patients that are observed ultimately require treatment.
Immunosuppresive agents such as sirolimus may also be effective (Tuberous Sclerosis)

26. Adenocarcinoma

27. RCC represents over 90% of all malignancies of the kidney in adults
Male:Female ratio is 2:1
It predominantly in the sixth to eighth decade of life with median age at diagnosis around 64 years of age
Third most common malignity of the Urinary System
Highly vascular
Not grossly infiltrative, except some collecting duct RCC and some sarcomatoid variants

28. Risk Factors Established
Tobacco exposure - Increases risk by about 50% in men and 20% in women
Obesity - Increases 24% for men and 34% for women for every 5 kg/m2 increase in BMI
Hypertension
Genetic factors - Von Hippel-Lindau (VHL) syndrome - heriditary papillary RCC - heriditary leiomyoma RCC - Birt-Hogg-Dube syndrome(BHD) - TS -ADPKD

29. Risk Factors Putative (generally considered to be) Lead compounds
Various chemicals (e.g., aromatic hydrocarbons)
Trichloroethylene exposure
Occupational exposure (metal, chemical, rubber, and printing industries)
Asbestos or cadmium exposure
CRF on dialysis and antihypertensive
Radiation therapy
Dietary (high fat/protein and lowfruits/vegetables)

30. Etiology Cause is unknown
A number of environmental and genetic factors have been studied as possible causes for renal cell carcinoma (RCC).

32. Pathology Originates from renal tubular epithelium
Equal frequency in both kidneys, randomly distributed in upper and lower poles
From cortex, tends to grow toward perinephric tissue
Characteristic bulge or mass effect
Gross: Yellow to Orange
Abundance of lipids (particularly in clear cell type)

33. No true capsule Histologically:
May have pseudocapsule formed by Compressed Renal Parenchyma, Fibrous Tissue and Inflammatory Cells
Histologically:
Mostly mixed Adenoca containing Clear cells, Granular cells and Sarcomatoid -appearing cells

34. Classification of Renal Cell Carcinoma
Based on Morphology and Cytogenetic Characteristics:
Clear Cell (75-85%)
Papillary (chromophilic) (10-15%)
Chromophobe (5-10%)
Oncocytic (3-7%)
Collecting Duct (Bellini’s Duct) (very Rare)
Unclassified (<2%)

36. Clear Cell Renal Adenocarcinoma
Abundant Cytoplasm contains Cholesterol, Triglycerides, Glycogen and Lipids
From Proximal Tubule
Solid or Cystic (rarely)
Associated with von Hippel-Lindau disease
Poor prognosis assoc. with higher nuclear grade or presence of sarcomatoid pattern

38. Papillary (Chromophilic) Adenocarcinoma
Contain less Glycogen and Lipids
Originate from Proximal Tubules
Type 1
Stage I or II
Favorable Prognosis
Type 2
Stage III or IV
Aggressive Tumor, Poor Prognosis

39. Chromophobe Adenocarcinoma
Originate from Intercalated Cells of Collecting System
Lack characteristic Lipid and Glycogen of most RCC
Lower risk of disease progression and death compared to clear cell
Patients present at lower stage

40. Oncocytic RCC (Oncocytoma)
Pure oncocytes
Large well-differentiated neoplastic cells
Originate from Intercalated Cells of Collecting Ducts
Behave in Benign Fashion
Well encapsulated, rarely invasive or associated with metastases

41. Collecting Duct (Bellini’s Duct) tumors
Rare but aggressive
In younger patients
Irregular borders with extensive anaplasia
Likely to invade blood vessels and cause infarction of tissue
Commonly present with Gross Hematuria

42. Pathogenesis Vascular tumors Spread by
Direct invasion through renal capsule into perinephric fat and adjacent structures
Direct extension into renal vein
25-30% have distant metastasic disease at presentation
Most common site of distant metastasis: Lung
Others: Liver, Bone, Ipsilateral Adjacent Lymph Nodes, Adrenal Gland, Brain, Opposite Kidney, Subcutaneous Tissue

43. Clinical Presentation
Many remain asymptomatic until the late disease stages >%50
Propensity to present with manifold clinical signs, symptoms, and paraneoplastic syndromes on the basis of local tumor extent, distant spread, biological activity.
Can be true incidental tumors, classic triad symptoms, and constitutional symptoms (weight loss, fever, night sweats, anorexia, cough, malaise, etc.)

44. Classic triad (6-10%)
Unilateral flank pain
Hematuria
Palpable mass

45. Symptoms Secondary to Metastasis:
Hematuria (invasion of urinary tract)
Dyspnea
Cough
Bone Pain
Scrotal Varicoceles (Kidney Tm obstructing gonadal vein where it enters renal vein)
Lower extremity Edema, Ascites, Hepatic Dysfunction, Pulmonary Emboli  IVC involvement

46. Laboratory Findings
Anemia (30%)
Hematuria
Elevated ESR

47. Paraneoplastic syndromes
Erythrocytosis (3-4%)
Hypertension (22-38%)
Elevated human chorionic gonadotropin levels
Cushing syndrome
Hyperprolactinemia
Ectopic insulin and glucagon production
Raised alkaline phosphatase levels (10%)
Cachexia, weight loss (35% )
Anemia (21-41%)
Elevated sedimentation rate (50-60%)
Reversible hepatic dysfunction ( %)
Fever (7-17%)
Amyloidosis (3-5%)
Neuromyopathy (3%)
Hypercalcemia (3-6%)

48. Prognostic factors
Tumor related factors such as pathologic stage, tumor size, nuclear grade, and histologic subtype= independent
Patient related factors such as CKD and co-morbidity have a significant impact on overall survival
Clinical findings s/o compromised prognosis in presumed localized RCC
Symptomatic presentation
Weight loss of more than 10% of body weight
- Poor performance status
Other – molecular prognostic factors,

49. Diagnostic evaluation
Baseline workup
-LFT, KFT, Creatinine clearance, CBC, ESR, coagulation study, urinalysis , Renal scintigraphy
Essential workup
-CT Scan
Complimentary workup
-Ultrasound, MRI, PET, renal tumor biopsy

50. Ultrasonography Major criteria for a single simple cyst are:
The mass is round and sharply demarcated with smooth walls
No echoes (anechoic) within mass
Strong posterior wall echo indicating good sound transmission through the cyst

51. If US equivocal (complex cyst), or suggestive of malignancy
solid or complex
with internal echoes
and irregular walls
if calcifications or septae are seen
if multiple cysts are clustered so that they may be masking underlying carcinoma
PROCEED TO CT....

52. CT Imaging CT provides information on
- Function and morphology of the contralateral kidney
- Primary tumour extension;
- Venous involvement;
- Enlargement of locoregional LNs;
-Condition of the adrenal glands and other solid
organs
A typical finding of RCC
- heterogeneous pattern of enhancement

54. MRI

55. PET Scan

56. Renal angiogram

57. Intravenous pyelography

58. Differential Diagnosis
Simple Cyst (diagnosis by USG enough if asymptomatic)
Angiomyolipomas (low-attenuatiion areas produced by high fat content)
Renal Abscess (Fever, Flank Pain, Pyuria, Leukocytosis)
Benign Renal masses
Granulomas
Arteriovenous malformations
Renal Lymphoma
Transitional Cell Carcinoma of Renal Pelvis
Adrenal Cancer
Metastatic Disease (most commonly from lung or breast cancer)

59. Staging
Goal of staging: to select Appropriate Therapy and obtain Prognostic information
Clinical staging evaluation:
History
Physical examination
Complete Blood Count
Serum Chemistries (renal and hepatic function)
Urinanalysis
Chest x-ray / Chest CT
Abdominal and Pelvic CT
Radionuclide Bone scan

60. TNM system more accurate
Robson (1963)
Easy to use but not related directly to prognosis, no longer used
TNM system more accurate

64. Fuhrman grading
Most effective in Predicting Metastasis

65. Treatment of RCC Localized disease:
Partial Nephrectomy
Radical Nephrectomy: kidney, Gerota’s fascia, ipsilateral adrenal, proxial ½ of the ureter, lymph nodes
Partial Nephrectomy and wedge resection: tm<4 cm
Radical Nephrectomy is the gold standard treatment for localized RCC.

66. Disseminated Disease:
Surgery: cytoreductive nephrectomy (nephrectomy followed by IFN alpha)
Radiation therapy: effective palliation of metastatic disease to the brain, lungs, bone
Biologic Response Modifiers: IFN alpha
Targeted Therapy: not because they make the tumor shrink but instead increase the duration of tumor control
Bevacizumab: inactivates VEGF A
Anti-VEGF TKIs: sorafenib, sunitinib
mTOR inhibitors: Temsirolimus