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TUMOURS OF THE KIDNEY
SUNIL KUMAR.P
4/14/2018 1SUNIL KUMAR.P
TUMOUR
• Definition :
• An abnormal benign or malignant new growth
of tissue that possesses no physiological
function and arises from uncontrolled usually
rapid cellular proliferation.
4/14/2018 2SUNIL KUMAR.P
TYPES
• 1. BENIGN RENAL TUMOUR
• 2. MALIGNANT RENAL TUMOUR
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BENIGN TUMOUR
• DEFINITION :
• A benign tumor is a mass of cells (tumor) that
lacks the ability to invade neighboring tissue
or metastasize. These do not spread into, or
invade, nearby tissues however they can
sometimes be quite large.
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BENIGN RENAL TUMOURS
• A benign tumor is not a malignant tumor, which is
cancer.
• It does not invade nearby tissue or spread to other
parts of the body the way cancer can.
• In most cases, the outlook with benign tumors is
very good.
• But benign tumors can be serious if they press on
vital structures such as blood vessels or nerves.
• As there is universal increase in the use of
radiological imaging, benign renal masses are being
detected more frequently4/14/2018 5SUNIL KUMAR.P
MALIGNANT TUMOUR
• DEFINITION:
• A tumor that invades surrounding tissues, is
usually capable of producing metastases, may
recur after attempted removal, and is likely to
cause death unless adequately treated.
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INTRODUCTION
• Both benign and malignant tumours occur in the
kidney, the latter being more common.
• These may arise from………….
• Renal tubules
• 1.Adenoma,
2. Adenocarcinoma
• From Embryonic tissue……
• 1.Mesoblastic nephroma
• 2.Wilm’s Tumour(Nephroblastoma)
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• From mesenchymal tissue……
• 1.Angiomyolipoma
• 2.Medullary interstitial tumour
• From Epithelium of Renal pelvis……
• 1.Urothelial carcinoma
• Besides these tumours, the kidney may be the
site of the secondary tumours
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CLASSIFICATION OF THE KIDNEY
TUMOURS
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BENIGN KIDNEY TUMOURS
• Benign tumours are usually small and are
often an incidental finding at autopsy or
nephrectomy
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1. Cortical Adenoma
• Cortical tubular adenomas are common than
other benign renal neoplasm's.
• They are frequently multiple and associated
with chronic Pyelonephritis or benign
nephrosclerosis.
4/14/2018 11SUNIL KUMAR.P
• Asymptomatic .
• Despite the classification of adenoma as a
benign tumor, difficult to differentiate from
renal carcinoma.
• Unknown etiology .
• Small and slow growing .
• Look very much like malignant RCCs.
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• Gross :
• These tumours may form tiny nodules up to 3
CM in diameter.
• They are encapsulated and white or yellow.
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• Microscopic :
• They are composed of tubular cords or papillary
structures projecting into cystic space.
• The cells of adenoma usually uniform, cuboidal
with no atypicality or mitosis.
• Size of the tumour rather than histologic criteria
is considered more significant parameter….
• Tumour larger than 3 cm diameter are potentially
malignant and metastasising.
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MICROSCOPIC H & E
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• Oncocytoma is a benign epithelial tumour arising
from the collecting ducts.
• Benign, unifocal renal tumor that averages 5 to 8 cm
in diameter but can reach larger sizes;
• however, the presence of malignant elements has
been known to occur.
• More often in men than women .
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• Unknown etiology .
• Found incidentally .
• Partial or radical nephrectomy is the safest
method of treatment because of unreliability
of differentiating it from RCC preoperatively
4/14/2018 18SUNIL KUMAR.P
• Grossly :
• The tumour is encapsulated and has variable
size.
• C/s …. Is Homogeneous and has characteristic
mahpgany-brown or tan colour.
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• Microscopic :
• The tumour cells are plump with…….
• Abundant
• Finely granular
• Acidophilic cytoplasm and
• Round Nuclei
• Electron microscopy demonstrates numerous
mitochondria in the cytoplasm.
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• Unusual tumors of the kidney .
• Often but not always associated with
TUBEROUS,SCLEROSIS .
• Histologically, they are composed of fat cells,
blood vessels, and sheets of smooth muscle
cells.
• Profuse internal hemorrhage can occur
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• Malignant elements are present in about one
quarter of them and may lead to metastasis .
• Asymptomatic lesions smaller than 4 cm
should be followed with yearly ultrasounds.
• Larger tumors should be considered for
embolization or renal-sparing surgery.
• Conservative surgical therapy is necessary
because of frequent bilaterality and
multiplicity.
4/14/2018 25SUNIL KUMAR.P
GROSS & MICROSCOPC
4/14/2018 26SUNIL KUMAR.P
• Rare .
• Small .
• Typically found in smooth muscle –containing
areas of the kidney including renal capsule
and renal pelvis .
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• Seen in middle aged females .
• Best detected radio-logically by CT scan . ( due
to high fat content .)
4/14/2018 30SUNIL KUMAR.P
4/14/2018 31SUNIL KUMAR.P
• Very small in size ( average 0.5 cm ) or less
• Asymptomatic .
• More commonly found in women than men.
• Usually occur on the rim of the kidney
• May reach large size before being detected
• Because they can’t be distinguished from
malignant growths, they are often removed
through partial or complete nephrectomy .
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4/14/2018 33SUNIL KUMAR.P
4/14/2018 34SUNIL KUMAR.P
4/14/2018 35SUNIL KUMAR.P
• PEADIATRIC RENAL TUMORS
• Wilm’s tumor ( nephroblastoma ) .
• RENAL TUMORS IN ADULTS
• Hypernephroma adenocarcinoma(RCC)
• Transitional cell tumor (TCC) of renal pelvis
• Squamous cell carcinoma
4/14/2018 36SUNIL KUMAR.P
4/14/2018 37SUNIL KUMAR.P
• WILM’S TUMOUR
• RHABDOMYOMA
• CLEAR CELL CARCINOMA
• MESOBLASTIC NEPHROMA
4/14/2018 38SUNIL KUMAR.P
4/14/2018 39SUNIL KUMAR.P
CONTENTS…………
• Introduction
• Epidemiology
• Etiology
• Pathogenesis
• Clinical presentation
• Morphological features
• Diagnosis
• Treatment
• Prognosis…….
4/14/2018 40SUNIL KUMAR.P
• INTRODUCTION
• Wilms tumor (also called Wilms' tumor or
nephroblastoma) is a type of cancer that starts in
the kidneys.
• It is the most common type of kidney cancer in
children.
• It is named after Max Wilms, a German doctor
who wrote one of the first medical articles about
the disease in 1899.
4/14/2018 41SUNIL KUMAR.P
• Nephroblastoma or Wilm’s tumour is an
embryonic tumour derived from primitive
renal epithelial and mesenchymal
components.
• It is the most common abdominal malignant
tumour of young children b/w 1- 6 yr of age
with equal sex incidence.
4/14/2018 42SUNIL KUMAR.P
Epidemiology
• Second most common pediatric abdominal tumor and
• most common renal malignancy
• Incidence of Wilms’ tumor is 8 cases per million
• children under age 15(1-6 yrs)
• About 500 new cases per year
• Accounts for 6% of all childhood malignant tumors
• Presents between age 1-5; most commonly age 5
• 66% before age 5
• 95% before age 10
4/14/2018 43SUNIL KUMAR.P
ETIOLOGY & PATHOGENESIS
• Wilm’s tumor is thought to be caused by
alterations of genes responsible for normal
genitourinary development.
• Examples of common congenital anomalies
associated with Wilms tumor are , double
collecting system, horseshoe kidney, and
hypospadias.
• Environmental exposures, although considered,
seem relatively unlikely to play a role.
4/14/2018 44SUNIL KUMAR.P
• Wilm’s tumour has following etiologic associations
…………
• 1. A defect in chromosome 11 p 13 results in abnormal
growth of metanephric blastema without
differentiation into normal tubules and glomeruli.
• 2. A higher incidence has been seen in monozygotic
twins & cases with family history.
• 3.Asociation of wilm’s tumour with some other
congenital anomalies has been observed, especially of
the genitourinary tract.
• 4.A few other malignancies are known to have higher
incidence of wilm’s tumour. These include …..
4/14/2018 45SUNIL KUMAR.P
• - osteosarcoma
• - Botyroid sarcoma
• - Retinoblastoma
• - neuroblastoma etc…..
4/14/2018 46SUNIL KUMAR.P
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4/14/2018 SUNIL KUMAR.P 48
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4/14/2018 51SUNIL KUMAR.P
Nephrogenic rest
• Nephrogenic rest: A fragment of embryonic tissue in
the kidney retained after the period of embryonic
development.
• Nephrogenic rests are precursors (forerunners) of
Wilms tumor.
• Two distinct categories of nephrogenic rest are
recognized on the basis of their topographical
relation to the renal lobe.
•
4/14/2018 SUNIL KUMAR.P 52
• Intralobar nephrogenic rests occur within the
renal lobe and develop as the result of events
early in renal development.
• Pelilobar nephrogenic rests are confined to
the periphery of the renal lobe and are related
to late kidney development.
4/14/2018 SUNIL KUMAR.P 53
Presentation
• No tumor-specific symptoms
• 1/3rd patients may have anorexia, vomiting, malaise
• Most common presentation is painless abdominal mass
• Physical Exam
• Smooth, palpable large abdominal mass
• May reveal HTN
• Hematuria
• Associated congenital abnormalities
• Check labs – associated with Von Willebrand’s Disease
in up to 10% of cases
4/14/2018 54SUNIL KUMAR.P
4/14/2018 SUNIL KUMAR.P 55
Morphological Features
• Gross :
• The tumour is usually quite large, spheroidal, replacing
most of the kidney.
• It is generally solitary and unilateral but 5-10% cases
may have bilateral tumour.
• On C/s – the tumour shows characteristic variegated
appearance ,Soft fishsflesh-like grey-white to creamy –
yellow tumour with foci of necrosis and haemorrhages
and grossly identifiable myxomatous or cartilaginous
elements.
• Invasion into renal vein is grossly evident in half the
cases
4/14/2018 56SUNIL KUMAR.P
4/14/2018 SUNIL KUMAR.P 57
4/14/2018 SUNIL KUMAR.P 58
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• Microscopic Ex:
• Nephroblastoma shows mixture of primitive
epithelial and mesenchymal elements.
• Most of the tumour consist of small, round to
spindled, anaplastic, sarcomatoid tumour
cells.
• Mesenchymal elements such as smooth and
skeletal muscle, cartilage and bone, fat cells
and fibrous tissue, may be seen.
4/14/2018 60SUNIL KUMAR.P
4/14/2018 61SUNIL KUMAR.P
Diagnostic Considerations
• Conditions to be considered in the differential diagnosis of Wilms tumor
include the following:
• Mesoblastic nephroma - Most common renal tumor in the first month of
life
• Renal cell carcinoma
• Clear cell sarcoma of the kidney
• Rhabdoid tumor of the kidney
• Nonmalignant mass
• Hydronephrosis
• Multicystic kidney disease
• Renal cyst
• Renal thrombosis
• Dysplastic kidney
• Renal hemorrhage
4/14/2018 62SUNIL KUMAR.P
Laboratory Investigations…….
• Complete blood count (CBC)
• Chemistry profile - Including kidney function
tests and routine measurements of
electrolytes and calcium
• Urinalysis
• Coagulation studies
• Cytogenetics studies, including 1p and 16q
deletion
4/14/2018 63SUNIL KUMAR.P
Imaging Studies
• Four-field chest radiography
• Renal ultrasonography
• CT scanning
• MRI scanning
4/14/2018 64SUNIL KUMAR.P
Rx……….
4/14/2018 65SUNIL KUMAR.P
4/14/2018 66SUNIL KUMAR.P
Prognosis
• Approximately 80-90% of children with a
diagnosis of Wilms tumor survive with current
multimodality therapy.
• Patients who have tumors with favorable
histology have an overall survival rate of at
least 80% at 4 years after the initial diagnosis,
even in patients with stage IV disease.
• The 4-year relapse-free and overall survival
rates in patients with favorable-histology
4/14/2018 67SUNIL KUMAR.P
4/14/2018 68SUNIL KUMAR.P

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Tumors of the kidney

  • 1. TUMOURS OF THE KIDNEY SUNIL KUMAR.P 4/14/2018 1SUNIL KUMAR.P
  • 2. TUMOUR • Definition : • An abnormal benign or malignant new growth of tissue that possesses no physiological function and arises from uncontrolled usually rapid cellular proliferation. 4/14/2018 2SUNIL KUMAR.P
  • 3. TYPES • 1. BENIGN RENAL TUMOUR • 2. MALIGNANT RENAL TUMOUR 4/14/2018 3SUNIL KUMAR.P
  • 4. BENIGN TUMOUR • DEFINITION : • A benign tumor is a mass of cells (tumor) that lacks the ability to invade neighboring tissue or metastasize. These do not spread into, or invade, nearby tissues however they can sometimes be quite large. 4/14/2018 4SUNIL KUMAR.P
  • 5. BENIGN RENAL TUMOURS • A benign tumor is not a malignant tumor, which is cancer. • It does not invade nearby tissue or spread to other parts of the body the way cancer can. • In most cases, the outlook with benign tumors is very good. • But benign tumors can be serious if they press on vital structures such as blood vessels or nerves. • As there is universal increase in the use of radiological imaging, benign renal masses are being detected more frequently4/14/2018 5SUNIL KUMAR.P
  • 6. MALIGNANT TUMOUR • DEFINITION: • A tumor that invades surrounding tissues, is usually capable of producing metastases, may recur after attempted removal, and is likely to cause death unless adequately treated. 4/14/2018 6SUNIL KUMAR.P
  • 7. INTRODUCTION • Both benign and malignant tumours occur in the kidney, the latter being more common. • These may arise from…………. • Renal tubules • 1.Adenoma, 2. Adenocarcinoma • From Embryonic tissue…… • 1.Mesoblastic nephroma • 2.Wilm’s Tumour(Nephroblastoma) 4/14/2018 7SUNIL KUMAR.P
  • 8. • From mesenchymal tissue…… • 1.Angiomyolipoma • 2.Medullary interstitial tumour • From Epithelium of Renal pelvis…… • 1.Urothelial carcinoma • Besides these tumours, the kidney may be the site of the secondary tumours 4/14/2018 8SUNIL KUMAR.P
  • 9. CLASSIFICATION OF THE KIDNEY TUMOURS 4/14/2018 9SUNIL KUMAR.P
  • 10. BENIGN KIDNEY TUMOURS • Benign tumours are usually small and are often an incidental finding at autopsy or nephrectomy 4/14/2018 10SUNIL KUMAR.P
  • 11. 1. Cortical Adenoma • Cortical tubular adenomas are common than other benign renal neoplasm's. • They are frequently multiple and associated with chronic Pyelonephritis or benign nephrosclerosis. 4/14/2018 11SUNIL KUMAR.P
  • 12. • Asymptomatic . • Despite the classification of adenoma as a benign tumor, difficult to differentiate from renal carcinoma. • Unknown etiology . • Small and slow growing . • Look very much like malignant RCCs. 4/14/2018 12SUNIL KUMAR.P
  • 13. • Gross : • These tumours may form tiny nodules up to 3 CM in diameter. • They are encapsulated and white or yellow. 4/14/2018 13SUNIL KUMAR.P
  • 15. • Microscopic : • They are composed of tubular cords or papillary structures projecting into cystic space. • The cells of adenoma usually uniform, cuboidal with no atypicality or mitosis. • Size of the tumour rather than histologic criteria is considered more significant parameter…. • Tumour larger than 3 cm diameter are potentially malignant and metastasising. 4/14/2018 15SUNIL KUMAR.P
  • 16. MICROSCOPIC H & E 4/14/2018 16SUNIL KUMAR.P
  • 17. • Oncocytoma is a benign epithelial tumour arising from the collecting ducts. • Benign, unifocal renal tumor that averages 5 to 8 cm in diameter but can reach larger sizes; • however, the presence of malignant elements has been known to occur. • More often in men than women . 4/14/2018 17SUNIL KUMAR.P
  • 18. • Unknown etiology . • Found incidentally . • Partial or radical nephrectomy is the safest method of treatment because of unreliability of differentiating it from RCC preoperatively 4/14/2018 18SUNIL KUMAR.P
  • 19. • Grossly : • The tumour is encapsulated and has variable size. • C/s …. Is Homogeneous and has characteristic mahpgany-brown or tan colour. 4/14/2018 19SUNIL KUMAR.P
  • 21. • Microscopic : • The tumour cells are plump with……. • Abundant • Finely granular • Acidophilic cytoplasm and • Round Nuclei • Electron microscopy demonstrates numerous mitochondria in the cytoplasm. 4/14/2018 21SUNIL KUMAR.P
  • 23. • Unusual tumors of the kidney . • Often but not always associated with TUBEROUS,SCLEROSIS . • Histologically, they are composed of fat cells, blood vessels, and sheets of smooth muscle cells. • Profuse internal hemorrhage can occur 4/14/2018 23SUNIL KUMAR.P
  • 25. • Malignant elements are present in about one quarter of them and may lead to metastasis . • Asymptomatic lesions smaller than 4 cm should be followed with yearly ultrasounds. • Larger tumors should be considered for embolization or renal-sparing surgery. • Conservative surgical therapy is necessary because of frequent bilaterality and multiplicity. 4/14/2018 25SUNIL KUMAR.P
  • 26. GROSS & MICROSCOPC 4/14/2018 26SUNIL KUMAR.P
  • 27. • Rare . • Small . • Typically found in smooth muscle –containing areas of the kidney including renal capsule and renal pelvis . 4/14/2018 27SUNIL KUMAR.P
  • 30. • Seen in middle aged females . • Best detected radio-logically by CT scan . ( due to high fat content .) 4/14/2018 30SUNIL KUMAR.P
  • 32. • Very small in size ( average 0.5 cm ) or less • Asymptomatic . • More commonly found in women than men. • Usually occur on the rim of the kidney • May reach large size before being detected • Because they can’t be distinguished from malignant growths, they are often removed through partial or complete nephrectomy . 4/14/2018 32SUNIL KUMAR.P
  • 36. • PEADIATRIC RENAL TUMORS • Wilm’s tumor ( nephroblastoma ) . • RENAL TUMORS IN ADULTS • Hypernephroma adenocarcinoma(RCC) • Transitional cell tumor (TCC) of renal pelvis • Squamous cell carcinoma 4/14/2018 36SUNIL KUMAR.P
  • 38. • WILM’S TUMOUR • RHABDOMYOMA • CLEAR CELL CARCINOMA • MESOBLASTIC NEPHROMA 4/14/2018 38SUNIL KUMAR.P
  • 40. CONTENTS………… • Introduction • Epidemiology • Etiology • Pathogenesis • Clinical presentation • Morphological features • Diagnosis • Treatment • Prognosis……. 4/14/2018 40SUNIL KUMAR.P
  • 41. • INTRODUCTION • Wilms tumor (also called Wilms' tumor or nephroblastoma) is a type of cancer that starts in the kidneys. • It is the most common type of kidney cancer in children. • It is named after Max Wilms, a German doctor who wrote one of the first medical articles about the disease in 1899. 4/14/2018 41SUNIL KUMAR.P
  • 42. • Nephroblastoma or Wilm’s tumour is an embryonic tumour derived from primitive renal epithelial and mesenchymal components. • It is the most common abdominal malignant tumour of young children b/w 1- 6 yr of age with equal sex incidence. 4/14/2018 42SUNIL KUMAR.P
  • 43. Epidemiology • Second most common pediatric abdominal tumor and • most common renal malignancy • Incidence of Wilms’ tumor is 8 cases per million • children under age 15(1-6 yrs) • About 500 new cases per year • Accounts for 6% of all childhood malignant tumors • Presents between age 1-5; most commonly age 5 • 66% before age 5 • 95% before age 10 4/14/2018 43SUNIL KUMAR.P
  • 44. ETIOLOGY & PATHOGENESIS • Wilm’s tumor is thought to be caused by alterations of genes responsible for normal genitourinary development. • Examples of common congenital anomalies associated with Wilms tumor are , double collecting system, horseshoe kidney, and hypospadias. • Environmental exposures, although considered, seem relatively unlikely to play a role. 4/14/2018 44SUNIL KUMAR.P
  • 45. • Wilm’s tumour has following etiologic associations ………… • 1. A defect in chromosome 11 p 13 results in abnormal growth of metanephric blastema without differentiation into normal tubules and glomeruli. • 2. A higher incidence has been seen in monozygotic twins & cases with family history. • 3.Asociation of wilm’s tumour with some other congenital anomalies has been observed, especially of the genitourinary tract. • 4.A few other malignancies are known to have higher incidence of wilm’s tumour. These include ….. 4/14/2018 45SUNIL KUMAR.P
  • 46. • - osteosarcoma • - Botyroid sarcoma • - Retinoblastoma • - neuroblastoma etc….. 4/14/2018 46SUNIL KUMAR.P
  • 52. Nephrogenic rest • Nephrogenic rest: A fragment of embryonic tissue in the kidney retained after the period of embryonic development. • Nephrogenic rests are precursors (forerunners) of Wilms tumor. • Two distinct categories of nephrogenic rest are recognized on the basis of their topographical relation to the renal lobe. • 4/14/2018 SUNIL KUMAR.P 52
  • 53. • Intralobar nephrogenic rests occur within the renal lobe and develop as the result of events early in renal development. • Pelilobar nephrogenic rests are confined to the periphery of the renal lobe and are related to late kidney development. 4/14/2018 SUNIL KUMAR.P 53
  • 54. Presentation • No tumor-specific symptoms • 1/3rd patients may have anorexia, vomiting, malaise • Most common presentation is painless abdominal mass • Physical Exam • Smooth, palpable large abdominal mass • May reveal HTN • Hematuria • Associated congenital abnormalities • Check labs – associated with Von Willebrand’s Disease in up to 10% of cases 4/14/2018 54SUNIL KUMAR.P
  • 56. Morphological Features • Gross : • The tumour is usually quite large, spheroidal, replacing most of the kidney. • It is generally solitary and unilateral but 5-10% cases may have bilateral tumour. • On C/s – the tumour shows characteristic variegated appearance ,Soft fishsflesh-like grey-white to creamy – yellow tumour with foci of necrosis and haemorrhages and grossly identifiable myxomatous or cartilaginous elements. • Invasion into renal vein is grossly evident in half the cases 4/14/2018 56SUNIL KUMAR.P
  • 60. • Microscopic Ex: • Nephroblastoma shows mixture of primitive epithelial and mesenchymal elements. • Most of the tumour consist of small, round to spindled, anaplastic, sarcomatoid tumour cells. • Mesenchymal elements such as smooth and skeletal muscle, cartilage and bone, fat cells and fibrous tissue, may be seen. 4/14/2018 60SUNIL KUMAR.P
  • 62. Diagnostic Considerations • Conditions to be considered in the differential diagnosis of Wilms tumor include the following: • Mesoblastic nephroma - Most common renal tumor in the first month of life • Renal cell carcinoma • Clear cell sarcoma of the kidney • Rhabdoid tumor of the kidney • Nonmalignant mass • Hydronephrosis • Multicystic kidney disease • Renal cyst • Renal thrombosis • Dysplastic kidney • Renal hemorrhage 4/14/2018 62SUNIL KUMAR.P
  • 63. Laboratory Investigations……. • Complete blood count (CBC) • Chemistry profile - Including kidney function tests and routine measurements of electrolytes and calcium • Urinalysis • Coagulation studies • Cytogenetics studies, including 1p and 16q deletion 4/14/2018 63SUNIL KUMAR.P
  • 64. Imaging Studies • Four-field chest radiography • Renal ultrasonography • CT scanning • MRI scanning 4/14/2018 64SUNIL KUMAR.P
  • 67. Prognosis • Approximately 80-90% of children with a diagnosis of Wilms tumor survive with current multimodality therapy. • Patients who have tumors with favorable histology have an overall survival rate of at least 80% at 4 years after the initial diagnosis, even in patients with stage IV disease. • The 4-year relapse-free and overall survival rates in patients with favorable-histology 4/14/2018 67SUNIL KUMAR.P