KidneyTumors for II year MBBS students.ppt

Target audience: For II year MBBS students

1. Kidney TumorsKidney Tumors
Dr.CSBR.Prasad, MD., MNAMS.,
Aug-2024-CSBRP

2. Malignant TumorsMalignant Tumors
Aug-2024-CSBRP

3. Wilms’ tumorWilms’ tumor
[[Nephroblastoma]]
•Malignant tumor of embryonic nephrogenic
elements
•Composed of: 3 components
1.Blastema
2.Stromal elements
3.Epithelial elements
•Most common abdominal solid tumor in
children [1:10,000]
•80% of all pediatric neoplasms
Aug-2024-CSBRP

4. Wilms’ tumorWilms’ tumor
[[Nephroblastoma]]
PathogenesisPathogenesis:
Defects in WT1 gene (chr# 11)
•Protein is a transcription factors that regulates the
expression of genes Eg: IGF1, PDGF
Defect in WTX on X-chromosome
Associated with THREE congenital syndromes:
1.WAGR syndrome (PAX & WT1, rarely WT2)
2.Denys-Drash Syndrome (Intersex, mesangeal
sclerosis)
3.Beckwith-Weidmann syndrome (WT2)
Aug-2024-CSBRP

5. Wilms’ tumorWilms’ tumor
[[Nephroblastoma]]
PathologyPathology:
•Gross:
–Large
–Capsulated
–Bulging, grey white cut surface
•Histology: Three elements in varying proportions
1.Blastemal cells – small blue cells, arranged in trabaculae &
nests
2.Epitheial elements: Tubules, glomeruloid strucutes
3.Stromal elements: Smooth muscle, skeletal muscle and
fibroblasts
Aug-2024-CSBRP

6. Wilms’ tumorWilms’ tumor
[[Nephroblastoma]]
Clinical featuresClinical features:
•Between 1-3 years
•Abdominal mass
•Abdominal pain
•Hypertension
•Hematuria
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14. Syndromes associated with Wilms’
Denys–Drash syndrome
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15. Syndromes associated with Wilms’
WAGR syndrome
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Wilms’ tumor
Aniridia
Gonadoblastoma
Retardation

16. Syndromes associated with Wilms’
Beckwith–Wiedemann syndrome
Aug-2024-CSBRP

17. Wilms’ tumorWilms’ tumor
[[Nephroblastoma]]
PrognosisPrognosis:
•Good <2years of age
•Surgery, radiation and chemo forms
mainstay of therapy
•Long term survival >90%
Aug-2024-CSBRP

18. Renal Cell CarcinomaRenal Cell Carcinoma
Aug-2024-CSBRP

19. Renal Cell CarcinomaRenal Cell Carcinoma
•Malignant tumor of renal tubular cells
•Variety:
–Sporadic (most common)
–Hereditary (5%, bilateral, young age)
•Associated with THREE syndromes:
1.von Hipple-Lindau syndrome
2.AD RCC
3.Hereditary papillary RCC (inherited)
•Smoking
Aug-2024-CSBRP

20. von Hippel–Lindau disease
•Angiomatosis
•Hemangioblastoma
•Pheochromocytoma
•Papillary cystadenoma of epidydimis /
broad ligament
•Endolymphatic sac tumor
•RCC
Aug-2024-CSBRP

21. Renal cell
carcinoma
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22. Renal cell carcinomaRenal cell carcinoma
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23. Renal cell carcinoma - Renal cell carcinoma - Clear cell type
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24. Aug-2024-CSBRP
Renal cell carcinoma - Renal cell carcinoma - Clear cell type

25. RCC- Chromophobe typeRCC- Chromophobe type
•3rd most common subtype of RCC
•Morphological types:
1.“Classic" and
2.Eosinophilic types
•overlaps with oncocytoma and often poses a
diagnostic problem
•Cytogenetics: multiple chromosomal
losses involving 1, 6, 10, 13, 17, 21 and Y
(more than in oncocytoma)
Aug-2024-CSBRP

26. RCC- Chromophobe typeRCC- Chromophobe type
Gross:
–Well-circumscribed,
solid, beige or light
brown 
–Eosinophilic type can
be mahogany-brown
and ~1/5 has central
scar (similar to
oncocytoma)
Aug-2024-CSBRP

27. RCC- Chromophobe typeRCC- Chromophobe type
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28. RCC- Chromophobe typeRCC- Chromophobe type
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29. RCC- Chromophobe typeRCC- Chromophobe type
Colloidal iron stain
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30. RCC- Chromophobe typeRCC- Chromophobe type
Chromophobe is less common than
papillary, is indolent, is less aggressive, and
it progresses more slowly than the other
But, when it is metastatic, it is incurable
Aug-2024-CSBRP

31. RCC- Chromophobe typeRCC- Chromophobe type
•Inherited syndrome associated with
Chromophobe-RCC is Birt-Hogg-Dube
syndrome
•Mutation in BHD genes
Aug-2024-CSBRP

32. Birt–Hogg–Dubé syndrome
Aug-2024-CSBRP

33. Papillary – Type-1 RCCPapillary – Type-1 RCC
•Inherited syndrome:
–Germ line mutation of c-met gene
–Constitutive activation of c-met receptor
•In sporadic tumors: 10-15% c-met
mutation
Aug-2024-CSBRP

34. Papillary – Type-1 RCCPapillary – Type-1 RCC
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37. Papillary renal cell carcinoma
psammoma bodies
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38. Papillary – Type-2 RCCPapillary – Type-2 RCC
•Inherited syndrome:
–Germ line mutation of tumor suppressor gene
- Fumarate hydratase gene (HLRCC)
–Constitutive activation of c-met receptor
•In sporadic tumors:
–Not well characterized
Aug-2024-CSBRP

39. Papillary – Type-2 RCCPapillary – Type-2 RCC
Aug-2024-CSBRP

40. Collecting Duct carcinomaCollecting Duct carcinoma
[Bellini duct carcinoma]
•Aggressive epithelial malignancy of renal medulla
•Rare: <1% of adult renal epithelial tumors, controversial
– often a diagnosis of exclusion
•2/3 male, mean age 55 years, associated with analgesic
nephropathy 
•Origin: likely from distal collecting (Bellini’s) ducts
•Histology: Mixed features of RCC + TCC
Marked tumor desmoplasia
•Behavior: Like TCC
•Invariably presents at an advanced state
Aug-2024-CSBRP

41. Collecting Duct carcinomaCollecting Duct carcinoma
Gross:
•Infiltrative, firm, gray-white, mean 5 cm
•Originates in medulla (as do some clear cell
carcinomas)
•May have intrarenal metastases; usually no
hemorrhage
Aug-2024-CSBRP

42. Collecting Duct carcinomaCollecting Duct carcinoma
•Histology: Shows irregular tubules, high-grade hobnail cells
and marked desmoplasia
•Infiltrative borders
•Major criteria: involvement of medullary pyramid (small
tumors), irregular tubular architecture, marked
desmoplasia, high grade hobnail cells, positive for high
molecular weight cytokeratin and Ulex europaeus, no
urothelial carcinoma elsewhere
•Minor criteria: central location (large tumors), papillary
architecture with wide fibrous stalks and desmoplastic
stroma, inflammatory stroma with neutrophils; extensive
renal, extrarenal and vascular infiltration, mucin positive
Aug-2024-CSBRP

43. Collecting Duct carcinomaCollecting Duct carcinoma
Aug-2024-CSBRP

44. Collecting Duct carcinomaCollecting Duct carcinoma
Aug-2024-CSBRP

45. Collecting Duct carcinomaCollecting Duct carcinoma
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46. Renal medullary RCCRenal medullary RCC
•Renal medullary RCC is very closely
associated with collecting duct, and affects
young African Americans with Sickle cell
trait and sickle cell disease
Aug-2024-CSBRP

47. Renal medullary RCCRenal medullary RCC
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50. Renal tumors associated with
Sickle cell trait / disease
•Renal medullary RCC
•ALK positive RCC
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51. Sarcomatoid RCCSarcomatoid RCC
•It is associated with both clear cell and
non-clear cell RCC histologies
•Prognosis is relatively poor
Aug-2024-CSBRP

52. Sarcomatoid renal cell carcinoma. The tumor is infiltrative with
extensive necrosis; the sarcomatoid component is indicated by the
fleshy gray-white areas (top left part of the tumor).
Aug-2024-CSBRP

53. RCC - Sarcomatoid carcinomaRCC - Sarcomatoid carcinoma
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54. Chromophobe renal cell carcinoma with
sarcomatoid differentiation
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55. RCC - Sarcomatoid carcinomaRCC - Sarcomatoid carcinoma
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57. Points regarding “Bone
mets”
BK.Patil
•Osteosclerotic mets: Prostate, Breast,
Carcinoid
•Osteolytic mets: Most of the other mets are
osteolytic
Aug-2024-CSBRP

58. Points regarding “Bone
mets”
Bone seeking kidney tumor
“Clear cell sarcoma of kidney”
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59. Chest CT scan, an abdominal CT that shows a large kidney mass,
and a bone scan with widespread metastasis
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60. Acute LEFT
varicocele
"bag of worms"
surrounding the
testis
Think of RCC of left
kidney
Aug-2024-CSBRP

61. Mechanism of Acute left
varicocele in RCC
Left testicular vein
drains into left renal
vein; Where as right
testicular vein joins
IVC
RCC has propensity to
invade into veins and
may grow as a column
upto the right side of
the heartAug-2024-CSBRP

62. Name some childhood tumors?
Add ‘BLASTOMA’ to the tissue
•Medulloblastoma
•Retinoblastoma
•Nephroblastoma
•Hepatoblastoma
Aug-2024-CSBRP
Avoid:
Pulmonary blastoma
Osteoblastoma
Chondroblastoma

63. Summary
Two renal tumors of importance are:
Renal cell carcinoma
•Adults
•Presents with silent
hematuria
•Gross:
–Circumscribed, bright
yellow tumor
–One pole of the kidney
•Histology:
–Clear cells
–Rich vascularity
•Variants: Clear cell,
Chromophobe, Papillary,
Sarcomtoid, Medullary
•Chromophobe has got good
prognosis
•Sarcomatoid carcinoma has
got worst prognosis
•Spread by Hematogenous
route
•Bone mets are common
–Pulsatile mets
Aug-2024-CSBRP

64. Summary
Two renal tumors of importance are:
Wilms’ tumor
•Children
•Present with abdominal
swelling, pain and hematuria
•Histology: Blastemal cells,
epithelial tubules and
mesenchymal cells
•Syndromes: WAGR, Denys-
Drash, Beckwith-
Wiedemann
•WT gene mutation
•Good prognosis:
–<2yrs of age
•Surgery, chemoradiation
is the main stay of Tx
Aug-2024-CSBRP

65. Questions
1.“Silent hematuria” is a feature, of which
kidney tumor?
2.Acute left sided varicocele should rise the
suspicion of …..?
3.Name the type of RCC seen in sickle cell trait
/ anemia ?
4.Name some bone metastasizing tumors?
5.Name the three syndromes associated with
Wilms’ tumor?
Aug-2024-CSBRP

66. E N D
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67. Name the syndrome?
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68. Aug-2024-CSBRP
Testicular tumor
Name the syndrome?

69. Aug-2024-CSBRP
Name the
syndrome?

70. Identify the syndrome
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71. Identify this kidney tumorIdentify this kidney tumor
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72. What this patient may be
suffering from?
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