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Nephroblastoma ,Neuroblastoma &
CNS tumours
By
Dr OLATUNYA OS
DEPT OF PAEDIATRICS
College of Medicine
Ekiti State University
• Introduction
• Epidemiology
• Aetiology & Genetics
• Pathology
• Diagnosis
• Treatment
OUTLINE
INTRODUCTION
• Nephroblastoma (wilms tumor, WT) is the most common
malignant neoplasm in the urinary tract of children
• It is an embryonal renal neoplasm in which blastemal,
stromal and epithelial cell types are present.
• It is thought to arise from the metanephric blasterma in
response to external stimuli during intra and extra-uterine
life
• WT represents about 6% of all childhood malignancies
• Reported incidence = 8 cases per million children/ year
• Here about 20 cases are seen / year
• 75% < 5 years
• Peak incidence = 2-3 years
• There is no sex predilection (F = M )
EPIDEMIOLOGY
• The exact aetiology is unknown
• Recent studies point to genetic predisposition
• Deletion of Xmosone implicated
• WT is sporadic in 95%; familial in 1-2%; bilateral in
5-10%
2% of WT associated with syndromes:
1. WAGR (WT, Aniridia, Genitourinary malformation,
mental Retardation)
2. Beckwith Wiedman syndrome (BWS) (Omphalocele,
macroglossia and visceromegaly)
3. Denys-Drash syndrome (WT, intersex disorder,
nephropathy)
4. Perlman syndrome (Overgrowth syndrome with mental
retardation)
5. Other associated anomalies seen in WT: Isolated
hemihypertrophy, hypospadias, undescended testes.
AETIOLOGY
• Children with such syndromes should be screened with ultrasound
every 6 months till 8 years of age.
• GENES involved in WT pathogenesis include:
* WT1 gene. Tumor suppressor gene located on 11p13; required for
normal kidney and gonadal development. Mutation of the gene seen
in WAGR, DDS, bilateral WT & 10% sporadic WT.
* WT2 gene. Located on 11p15; mutation in form of deletion or
overexpression is linked to familial WT and BWS.
* Additional WT loci include 16q, 1p, and p53 (located at 17p13.)
Mutation in these are associated with poor prognosis
• MACROSCOPY: WT is sharply demarcated and variably
encapsulated with haemorrhagic areas and cavitations.
Location may be central or polar
• MICROSCOPY: 3 components seen in normal kidney
differentiation--- blastema, tubules and stroma are present.
Based on microscopy, 2 broad variants are known:
1. Unfavorable histology. Presence of anaplasia– cells with
nuclear enlargement, hyperchromatic nuclei and abnormal
mitotic figures.
ANAPLASIA IS THE SINGLE MOST IMPORTANT INDICATOR
OF POOR PROGNOSIS.
2. Favorable histology. WT without anaplastic features.
PATHOLOGY
• STAGES. National Wilms Tumor Study Group NWTSG
staging system is widely used.
Stage I– Tumor limited to kidney and completely resected
II– Tumor extends beyond the kidney but is completely
excised
III– Residual non-haematogenous tumor confined to the
abdomen
IV– Haematogenous metastases
V– Bilateral tumor
STAGING
History: Commonest is asymptomatic abdominal mass. Others
include abdominal pain, fever, weight loss, haematuria,
anaemia, and varicocele.
WT may rupture and present as acute abdomen
Physical examination: Carefully palpate the abdomen, measure
the blood pressure, look into the eyes; and document
anomalies if present.
Investigations: Should define the nature of abdominal mass;
organ of origin; status of the contralateral kidney; state of the
vena cava; and distant metastases.
* Ultrasound
* IVU
* CT scan
* Chest X ray
* Others: S/E/U/C, Full blood count.
DIAGNOSIS
Involves surgical excision, post operative radiotherapy and chemotherapy.
This can be modified by: stage of disease, age of patient, size of tumor and
clinical state of patient.
Surgery:* serves to excise the tumor and determine intra abdominal stage.
* Important surgical caveats.
* Peculiar situations: IVC involvement, Bilateral tumors
*Complications: Haemorrhage, small bowel obstruction.
Chemotherapy:+ Mostly as adjuvant. Can be used as neoadjuvant in
inoperable tumor; non resectable tumor; bilateral tumor; and IVC
involvement.
+Agents: Actinomycin D, Vincristine, Adriamycin,
Cyclophosphamide.
TREATMENT
Radiotherapy:* Same indication as chemotherapy.
* Renal bed demarcated by titanium clips placed during
surgery.
Treatment by stage
a. Unfavorable histology. All modalities for all stages
b. Favorable histology. Stages I & II, < 2 years--- SURGERY
> 2 “ -- SURG.+ CHEMO.
Stages III,IV,V– All modalities.
Results: Stage I = 96.5%
II = 92.2%
III =73%
IV/V/all unfavorable histology =68.1%
INTRODUCTION
Tumor of neural crest origin
- May arise from the adrenal or the sympathetic ganglion from
neck to pelvis
- Most common solid tumor in childhood
- Spontaneous regression and tumor maturation observed in a
few cases
- Advances in management have not significantly improved
outcome
EPIDEMIOLOGY
* Incidence= 1 in 8,000-10,000 children.
* >50% are 2 years or less at diagnosis
* M > F 1.2 : 1.0
NEUROBLASTOMA
Sites: 75% -- Retroperitoneal
50% Adrenal medulla
25% Paraspinal ganglia
20% -- Posterior mediastinum
5% -- Neck and Pelvis
Macroscopy: - Purple, highly vascular and friable.
- Becomes nodular as it matures or responds to therapy.
Microscopy: - Composed of neuroblasts– small round cell with prominent nucleus
and small cytoplasm.
- Immature tumor has no special arrangement of cells
- More mature tumor show rosette formation; some may resemble
normal ganglion cells.
Electron microscopy shows neurofibrils and electron dense granules; this rules out
rhabdomyosarcoma and Ewing’s tumor
PATHOLOGY
Evans staging is commonly used.
Stage I - Tumor confined to the organ of origin
II - Tumor extends beyond the organ of origin but not crossing midline.
Ipsilateral nodes may be involved
III - Tumor extends beyond midline. Bilateral nodes may be involved
IV - Distant metastases
IVS- Same as I or II with presence of disease in the liver, skin or bone
marrow.
STAGING
Aetiology:
Unknown.
Hereditary factors important
Cytogenetics/ Molecular biology: Alterations seen in NB are
1. Mutation at 1p36 – associated with poor outcome
2. Mutation at 14q
3. Amplification of N-myc protooncogene– poor prognosis
4. Expression of Multidrug resistant related protein gene and
bcl-2 gene activity--- poor outcome.
5. Expression of Nerve growth factor recceptors– good
prognosis
AETIOLOGY
Presentation: Related to the site of primary tumor; Metastases; and Metabolic
tumor products.
Common presentations include abdominal mass; weight loss; FTT; and anaemia.
Others: Respiratory distress, dysphagia, paraplegia, Horners syndrome,
hypertension, flushing & iritability, urinary sympyoms, constipation, skin
nodules, ‘panda eye’, watery diarrhea, dancing-eye syndrome etc.
Investigations: 1. Plain Xray
2. Ultrasonography
3. IVU
4.CT scan
5. Bone scan with MIBG
6. Urine chemistry: HMA= Undifferentiated NB; VMA= More
mature NB.
7.Blood chemistry: NSE, LDH, Ferritin
8. Immunohistochemistry: Neurofilament, NSE etc
9. Biopsy & Bone marrow aspiration
Others: LFT, FBC.
PRESENTATION & DIAGNOSIS
1. VMA/ HVA
2. Lactate dehydrogenase
3. Serum ferritin
4. Neuron-Specific Enolase NSE
5. Tumor derived gangloside GD2
TUMOUR MARKERS
• Depends the stage at diagnosis
• Localized tumors are best managed with surgery
• Partially resected or unresectable tumors need CHEMO. & or RADIO.
• Surgery: Operative principles.
• Chemotherapy: Adjuvant or Neoadjuvant
Agents: Cyclophosphamide, Doxorubicin, Vincristine, Cisplatin.
Radiotherapy: For > 1 year olds. MIBG can be used.
Immunotherapy: IL-2 has been tried.
Treatment by stages
Stage I- SURG.
II- SURG + CHEMO
III- High CHEMO. then SURG.
IV- < 5 months- CHEMO then SURG
> 5 “ - High CHEMO or RAD. Then SURG, & High CHEMO
IVS- Supportive care
TREATMENT
Outcome: Stage I – 90%
Stage II --80%
Stage III --60%
Stage IV --10%
Stage IVS --70%
Future THINKING: 1. Early diagnosis. Mass screening for VMA & HVA at 6
months
2. Use of differentiating agents e.g.13 cis – retinoic acid
3.Targetted Immunotherapy with anti ganglioside GD2 antibodies
for advanced NB.
CENTRAL NERVOUS SYSTEM TUMOURS
Generally, clinical features may be insidious onset and include
1. Deteriorating school performance
2. Hand preference
3. Changing handwriting
4. Changing personality & child`s behaviour
5. Convulsion due to pressure effects/invasion of vital area
6. Features of raised intracranial pressure
7. May paradoxically presents with weight gain/obesity due to
Diencephalon syndrome
8. Visual/Hearing impairment
9. May present with Diabetes insipidous/DBM
10. May present with syndrome of inappropriate ADH synthesis
11. May present with stroke
CLASSIFICATION
Two broad groups are recognised
A. INFRATENTORIAL/POSTERIOR CRANIAL FOSSA TUMOURS
They are located within the posterior cranial fossa
Or have relationships with it examples include
1. Cerebellar Astrocytoma
2. Medulloblastoma
3. Brain Stem glioma
4. Ependymoma of the 4th ventricle = Most malignant of them
5. Acoustic neuroma & Meningioma: They are rare in kids
B. SUPRATENTORIAL TUMOURS
1. Craniopharygiomas
INVESTIGATIONS IN CNS TUMOURS
1. CT scan: Delienate the lesion
2. MRI
3. CSF Analysis
4. Contrast studies
5. SKULL X-RAY
a. May show calcification/enlargement of sellartursica craniopharyngioma
b. Calcification of 4th ventricle in Ependymoma
c. Evidence of raised intracranial pressure
6. OPTHMOLOGICAL EXAMINATION: Fundoscopy
7. OTHER STUDIES
a. Serum electrlyte = SIADH
b. Urinalysis & Electrolyte = SIADH
c. Other biochemical & blood assays
d. Cerebral angiography
SALIENT FEATURES OF SOME CNS TUMOURS
Cerebellar Astrocytoma
1. Highest icidence is between 2- 8years
2. Signs of raised ICP
3. Signs of cerebellar lesions:Nystagmus,Tremor,Ataxia,
4. Seizures
5. Usually there is lateralising signs
TREATMENT: Surgery + Chemo + Irradiation
Medulloblastoma
1. Midline in location
2. Fastest growing intracranial tumour
3. Boys more affected than girls
4. Clinical features similar toCerebellar astrocytoma but
5. without lateralising signs usually
TREATMENT: Surgery + chemo + irradiation
Salient clinical features contd
Ependymoma of 4th ventricule
1. Rapidly evolving signs of ICP are the major features
2. Cranial nerves palsies
3. +ve Babinskis sign may be seen due to brain stem infilteration
TREAMENT
Surgical excision + Irradiation + chemo
Glioma of the brain stem
1. Peak age is 6-8years
2. Progressive bilateral cranial nerve palsies: 6th&7thnerves commonly
3. Any other cranial nerve can be affected
4. Upper motor neuron lesions types : Hypertonia, Hyperreflexia etc
5. Ataxia
6. Usually does not present with features of raised ICP
TREATMENT
Not amenable to surgery only LOCAL IRRADIATION + chemo
SALIENT FEATURES CONTD
Craniopharygioma
Inicidence: Any age during childhood The tumour arises from
the squamous epithelia cells of the embryonal Ratchke`s pouch
Symptoms
1. Growth failure
2. Arrest of linear growth
3. Raised intra-cranial pressure
4. Diabetes insipidous
5. Visual impairments may include
a. Bitemporal hemianopsia: Optic chiasma lesion/involvement
b. Assymetric field defect
c. Decreased visual acuity
Treatment
Surgical excision
Use of steroids to shrink tumour before surgery & maintanance after
Tumour cyst aspiration + follow up RADIOTHERAPY
THANK YOU

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DR OLATUNYA NEPHROBLASTOMA & NEUROBLASTOMA LECTURE.pptx

  • 1. Nephroblastoma ,Neuroblastoma & CNS tumours By Dr OLATUNYA OS DEPT OF PAEDIATRICS College of Medicine Ekiti State University
  • 2. • Introduction • Epidemiology • Aetiology & Genetics • Pathology • Diagnosis • Treatment OUTLINE
  • 3. INTRODUCTION • Nephroblastoma (wilms tumor, WT) is the most common malignant neoplasm in the urinary tract of children • It is an embryonal renal neoplasm in which blastemal, stromal and epithelial cell types are present. • It is thought to arise from the metanephric blasterma in response to external stimuli during intra and extra-uterine life
  • 4. • WT represents about 6% of all childhood malignancies • Reported incidence = 8 cases per million children/ year • Here about 20 cases are seen / year • 75% < 5 years • Peak incidence = 2-3 years • There is no sex predilection (F = M ) EPIDEMIOLOGY
  • 5. • The exact aetiology is unknown • Recent studies point to genetic predisposition • Deletion of Xmosone implicated • WT is sporadic in 95%; familial in 1-2%; bilateral in 5-10% 2% of WT associated with syndromes: 1. WAGR (WT, Aniridia, Genitourinary malformation, mental Retardation) 2. Beckwith Wiedman syndrome (BWS) (Omphalocele, macroglossia and visceromegaly) 3. Denys-Drash syndrome (WT, intersex disorder, nephropathy) 4. Perlman syndrome (Overgrowth syndrome with mental retardation) 5. Other associated anomalies seen in WT: Isolated hemihypertrophy, hypospadias, undescended testes. AETIOLOGY
  • 6. • Children with such syndromes should be screened with ultrasound every 6 months till 8 years of age. • GENES involved in WT pathogenesis include: * WT1 gene. Tumor suppressor gene located on 11p13; required for normal kidney and gonadal development. Mutation of the gene seen in WAGR, DDS, bilateral WT & 10% sporadic WT. * WT2 gene. Located on 11p15; mutation in form of deletion or overexpression is linked to familial WT and BWS. * Additional WT loci include 16q, 1p, and p53 (located at 17p13.) Mutation in these are associated with poor prognosis
  • 7. • MACROSCOPY: WT is sharply demarcated and variably encapsulated with haemorrhagic areas and cavitations. Location may be central or polar • MICROSCOPY: 3 components seen in normal kidney differentiation--- blastema, tubules and stroma are present. Based on microscopy, 2 broad variants are known: 1. Unfavorable histology. Presence of anaplasia– cells with nuclear enlargement, hyperchromatic nuclei and abnormal mitotic figures. ANAPLASIA IS THE SINGLE MOST IMPORTANT INDICATOR OF POOR PROGNOSIS. 2. Favorable histology. WT without anaplastic features. PATHOLOGY
  • 8. • STAGES. National Wilms Tumor Study Group NWTSG staging system is widely used. Stage I– Tumor limited to kidney and completely resected II– Tumor extends beyond the kidney but is completely excised III– Residual non-haematogenous tumor confined to the abdomen IV– Haematogenous metastases V– Bilateral tumor STAGING
  • 9. History: Commonest is asymptomatic abdominal mass. Others include abdominal pain, fever, weight loss, haematuria, anaemia, and varicocele. WT may rupture and present as acute abdomen Physical examination: Carefully palpate the abdomen, measure the blood pressure, look into the eyes; and document anomalies if present. Investigations: Should define the nature of abdominal mass; organ of origin; status of the contralateral kidney; state of the vena cava; and distant metastases. * Ultrasound * IVU * CT scan * Chest X ray * Others: S/E/U/C, Full blood count. DIAGNOSIS
  • 10. Involves surgical excision, post operative radiotherapy and chemotherapy. This can be modified by: stage of disease, age of patient, size of tumor and clinical state of patient. Surgery:* serves to excise the tumor and determine intra abdominal stage. * Important surgical caveats. * Peculiar situations: IVC involvement, Bilateral tumors *Complications: Haemorrhage, small bowel obstruction. Chemotherapy:+ Mostly as adjuvant. Can be used as neoadjuvant in inoperable tumor; non resectable tumor; bilateral tumor; and IVC involvement. +Agents: Actinomycin D, Vincristine, Adriamycin, Cyclophosphamide. TREATMENT
  • 11. Radiotherapy:* Same indication as chemotherapy. * Renal bed demarcated by titanium clips placed during surgery. Treatment by stage a. Unfavorable histology. All modalities for all stages b. Favorable histology. Stages I & II, < 2 years--- SURGERY > 2 “ -- SURG.+ CHEMO. Stages III,IV,V– All modalities. Results: Stage I = 96.5% II = 92.2% III =73% IV/V/all unfavorable histology =68.1%
  • 12. INTRODUCTION Tumor of neural crest origin - May arise from the adrenal or the sympathetic ganglion from neck to pelvis - Most common solid tumor in childhood - Spontaneous regression and tumor maturation observed in a few cases - Advances in management have not significantly improved outcome EPIDEMIOLOGY * Incidence= 1 in 8,000-10,000 children. * >50% are 2 years or less at diagnosis * M > F 1.2 : 1.0 NEUROBLASTOMA
  • 13. Sites: 75% -- Retroperitoneal 50% Adrenal medulla 25% Paraspinal ganglia 20% -- Posterior mediastinum 5% -- Neck and Pelvis Macroscopy: - Purple, highly vascular and friable. - Becomes nodular as it matures or responds to therapy. Microscopy: - Composed of neuroblasts– small round cell with prominent nucleus and small cytoplasm. - Immature tumor has no special arrangement of cells - More mature tumor show rosette formation; some may resemble normal ganglion cells. Electron microscopy shows neurofibrils and electron dense granules; this rules out rhabdomyosarcoma and Ewing’s tumor PATHOLOGY
  • 14. Evans staging is commonly used. Stage I - Tumor confined to the organ of origin II - Tumor extends beyond the organ of origin but not crossing midline. Ipsilateral nodes may be involved III - Tumor extends beyond midline. Bilateral nodes may be involved IV - Distant metastases IVS- Same as I or II with presence of disease in the liver, skin or bone marrow. STAGING
  • 15. Aetiology: Unknown. Hereditary factors important Cytogenetics/ Molecular biology: Alterations seen in NB are 1. Mutation at 1p36 – associated with poor outcome 2. Mutation at 14q 3. Amplification of N-myc protooncogene– poor prognosis 4. Expression of Multidrug resistant related protein gene and bcl-2 gene activity--- poor outcome. 5. Expression of Nerve growth factor recceptors– good prognosis AETIOLOGY
  • 16. Presentation: Related to the site of primary tumor; Metastases; and Metabolic tumor products. Common presentations include abdominal mass; weight loss; FTT; and anaemia. Others: Respiratory distress, dysphagia, paraplegia, Horners syndrome, hypertension, flushing & iritability, urinary sympyoms, constipation, skin nodules, ‘panda eye’, watery diarrhea, dancing-eye syndrome etc. Investigations: 1. Plain Xray 2. Ultrasonography 3. IVU 4.CT scan 5. Bone scan with MIBG 6. Urine chemistry: HMA= Undifferentiated NB; VMA= More mature NB. 7.Blood chemistry: NSE, LDH, Ferritin 8. Immunohistochemistry: Neurofilament, NSE etc 9. Biopsy & Bone marrow aspiration Others: LFT, FBC. PRESENTATION & DIAGNOSIS
  • 17. 1. VMA/ HVA 2. Lactate dehydrogenase 3. Serum ferritin 4. Neuron-Specific Enolase NSE 5. Tumor derived gangloside GD2 TUMOUR MARKERS
  • 18. • Depends the stage at diagnosis • Localized tumors are best managed with surgery • Partially resected or unresectable tumors need CHEMO. & or RADIO. • Surgery: Operative principles. • Chemotherapy: Adjuvant or Neoadjuvant Agents: Cyclophosphamide, Doxorubicin, Vincristine, Cisplatin. Radiotherapy: For > 1 year olds. MIBG can be used. Immunotherapy: IL-2 has been tried. Treatment by stages Stage I- SURG. II- SURG + CHEMO III- High CHEMO. then SURG. IV- < 5 months- CHEMO then SURG > 5 “ - High CHEMO or RAD. Then SURG, & High CHEMO IVS- Supportive care TREATMENT
  • 19. Outcome: Stage I – 90% Stage II --80% Stage III --60% Stage IV --10% Stage IVS --70% Future THINKING: 1. Early diagnosis. Mass screening for VMA & HVA at 6 months 2. Use of differentiating agents e.g.13 cis – retinoic acid 3.Targetted Immunotherapy with anti ganglioside GD2 antibodies for advanced NB.
  • 20. CENTRAL NERVOUS SYSTEM TUMOURS Generally, clinical features may be insidious onset and include 1. Deteriorating school performance 2. Hand preference 3. Changing handwriting 4. Changing personality & child`s behaviour 5. Convulsion due to pressure effects/invasion of vital area 6. Features of raised intracranial pressure 7. May paradoxically presents with weight gain/obesity due to Diencephalon syndrome 8. Visual/Hearing impairment 9. May present with Diabetes insipidous/DBM 10. May present with syndrome of inappropriate ADH synthesis 11. May present with stroke
  • 21. CLASSIFICATION Two broad groups are recognised A. INFRATENTORIAL/POSTERIOR CRANIAL FOSSA TUMOURS They are located within the posterior cranial fossa Or have relationships with it examples include 1. Cerebellar Astrocytoma 2. Medulloblastoma 3. Brain Stem glioma 4. Ependymoma of the 4th ventricle = Most malignant of them 5. Acoustic neuroma & Meningioma: They are rare in kids B. SUPRATENTORIAL TUMOURS 1. Craniopharygiomas
  • 22. INVESTIGATIONS IN CNS TUMOURS 1. CT scan: Delienate the lesion 2. MRI 3. CSF Analysis 4. Contrast studies 5. SKULL X-RAY a. May show calcification/enlargement of sellartursica craniopharyngioma b. Calcification of 4th ventricle in Ependymoma c. Evidence of raised intracranial pressure 6. OPTHMOLOGICAL EXAMINATION: Fundoscopy 7. OTHER STUDIES a. Serum electrlyte = SIADH b. Urinalysis & Electrolyte = SIADH c. Other biochemical & blood assays d. Cerebral angiography
  • 23. SALIENT FEATURES OF SOME CNS TUMOURS Cerebellar Astrocytoma 1. Highest icidence is between 2- 8years 2. Signs of raised ICP 3. Signs of cerebellar lesions:Nystagmus,Tremor,Ataxia, 4. Seizures 5. Usually there is lateralising signs TREATMENT: Surgery + Chemo + Irradiation Medulloblastoma 1. Midline in location 2. Fastest growing intracranial tumour 3. Boys more affected than girls 4. Clinical features similar toCerebellar astrocytoma but 5. without lateralising signs usually TREATMENT: Surgery + chemo + irradiation
  • 24. Salient clinical features contd Ependymoma of 4th ventricule 1. Rapidly evolving signs of ICP are the major features 2. Cranial nerves palsies 3. +ve Babinskis sign may be seen due to brain stem infilteration TREAMENT Surgical excision + Irradiation + chemo Glioma of the brain stem 1. Peak age is 6-8years 2. Progressive bilateral cranial nerve palsies: 6th&7thnerves commonly 3. Any other cranial nerve can be affected 4. Upper motor neuron lesions types : Hypertonia, Hyperreflexia etc 5. Ataxia 6. Usually does not present with features of raised ICP TREATMENT Not amenable to surgery only LOCAL IRRADIATION + chemo
  • 25. SALIENT FEATURES CONTD Craniopharygioma Inicidence: Any age during childhood The tumour arises from the squamous epithelia cells of the embryonal Ratchke`s pouch Symptoms 1. Growth failure 2. Arrest of linear growth 3. Raised intra-cranial pressure 4. Diabetes insipidous 5. Visual impairments may include a. Bitemporal hemianopsia: Optic chiasma lesion/involvement b. Assymetric field defect c. Decreased visual acuity Treatment Surgical excision Use of steroids to shrink tumour before surgery & maintanance after Tumour cyst aspiration + follow up RADIOTHERAPY