1. Neuroblastoma and
Nephroblastoma
Dr. Kalpana Malla
MD Pediatrics
Manipal Teaching Hospital
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2. NEUROBLASTOMA (NB)
• Most common extracranial tumour
• 8% of childhood cancers.
• Commonest solid tumour
• Median age – 2yrs may present antenatally (
adrenal gland)
• 90% are < 5 yrs
• M>F

3. NEUROBLASTOMA (NB)
• Variable clinical presentation.
• May undergo spontaneous regression
Tumour secretes –
1. catecholamines- VMA,HVA
2. neurone specificenolase(NSE)
3 ferritin

4. NEUROBLASTOMA (NB)
• Pathology –
Small round cell tumour—variable
degree of differentiation
• Genetics: -
mycn proto-oncogene is seen amplified in 25
% of NB associated with advanced stage – rapid
progression and poor outcome.

5. Clinical Features
• Any site with Neural crest cell – post cranial
fossa to coccyx
• 70% in abdomen—in Adrenal gland 50%
• Retro-peritoneal ganglia 50%
• 20% in thorax: - poterior medisatinum

6. Clinical Features
• Infants: -
More localized –in cervical or
intrathoracic areas.
• Older : -
Abdomen with disseminated disease.

7. Clinical Features
• Appears chronically ill- Fever, irritability, FTT
• Bone pain
• Bluish subcutaneous nodules-blueberry muffins -
mainly in neonates and infants
• Periorbital ecchymosis – Racoon eye & proptosis

8. Clinical Features
• Epidural: -featurs of raised intracranial pressure
• Paraspinal –limb paresis
• Primary in nasopharynx - epistaxis
• Acute cerebellar encephalopathy- cerebellar ataxia
• Neural foramina: -
Spinal and Root compression

9. Clinical Features
• Opsoclonus-Myoclonus: " dancing eyes, dancing feet
syndrome"—better prognosis 4%
• Cervical involvement: -
Horner's syndrome
Thorax:-
Respiratory distress
• Abdomen: -
Hard mass, fixed, abdominal
Discomfort, Massive hepatomegaly

10. Clinical Features
• Vasoactive intestinal peptides -
diarrhoea, hypokalemia
• increased catecholamines -
flushing, tachycardia, inc
sweating,hypertension
• Metastatic: -
BM, bone, liver and skin.

11. Diagnosis:
• Hemoblobin ↓
• Platelets↓
• LDH 
• NSE 
• Urea, creatinine 
• Inc. HVA, VMA.

12. Imaging
• CXR
• USG
• CT
• MRI
• Bone scan

13. Confirm
• Tissue biopsy
• Bone marrow aspiration
•  urine & S. catecholamine
• DNA content- mycn amplification

14. STAGING: INSS
• Stage I- confined to organ/ structure.
• Stage II- extends beyond structure- does
not cross the midline.
• A – without Ipsilateral lymph node
involvement
• B – with Ipsilateral lymph node
involvement

15. STAGING: INSS
• Stage III- Bilateral lesions with/ without
bilateral lymph node involvement
• Stage IV – Disseminated to distant sites.
• IV s- Below 1 year of age with disseminated
disease to BM/Skin or liver
• Neonatal stage IVs -spontaneous remission

16. Treatment
• Surgical excision
• Chemotherapy—
• Cisplatin, Doxorubicin, Vincristine, cyclophos
phamide—may help to convert unresectable
tumour into a resectable one
• Radiotherapy

17. Prognosis—3 year survival
95% 25-50% < 25%
Age < 1yr > 1yr 1-5 yrs
INSS 1,2,4s 3,4 3,4
mycn N N Amplified

18. Risk Directed: -
• Low risk—
Infants with 4s, Stage I INSS
- Surgery alone
2A,B or 3 < 1yr
—Surgery and Chemotherapy
• Intermediate risk: -
II B > 1yr
—Surgery+ Chemo

19. Intermediate risk: -
• Intermediate risk: -
stage – III
– Chemo+Surgery+ Radiation
stage IV < 1 yr
- Surgery followed by Chemo
• High risk —IV > 1yr
—Chemo+ Surgery+ BMT

20. Metastasis
• Local invasion- most common.invades
surrounding tissue
• Lymphatics – regional lymph nodes
• Hematogenous – bone marrow,skeleton and
liver
• Rare- brain,lungs

22. WILM'S TUMOUR—
NEPHROBLASTOMA
• Most common renal tumour
• Any part of either kidney- Solid growth, Sharply
demarcated,variably encapsulate
• Small areas of haemorrhage. Distorted renal
parenchyma with compression of renal tissue.

23. Histology: -
• Favourable histology
- Triphasic
- epithelial
- blastemal
- stromal cells.
• Unfavourable histology
- Anaplastic—10% of cases—60% deaths.
- Rhabdoid – found in very young patients.
- Clear cell Sarcoma- male predominance

24. STAGING – National W T Study
Stage I-
Limited to kidney, fully resectable with capsule
intact.
Stage II
Extends beyond kidney, fully resectable.
Stage III
Post surgical residual, non-haematogenous
extension confined to abdomen.

25. STAGING – National W T Study
• Stage IV
Haematological metastasis—often
to the lung.
• Stage V
Bilateral renal disease

26. Clinical Features
• Median age 3years
• M=F
• Looks less ill Abd/ flank mass—aymptomatic
• Smooth, firm, rarely cross midline.
• Discovered by chance.
• 50% have abdominal pain, vomiting or both.
• 60% have hypertension due to renal ischaemia.
Haematuria.

27. Syndromes
• WAGR –
WT, Aniridia, Genitourinary
malformations, Mental Retardation. Chr
deletion 11 p 13
• Beckwith Wiedemann –
Organomegaly, Macroglossia, omphalocoele, he
mihypertrophy. Chr deletion-11p15

28. Syndromes
• Deny's Drasch—
WT, Nephropathy, Genital abnormalities.
• Familial WT
• Perlman syndrome
• Paraneoplastic syndrome-
Inc Erythropoeitin with Polycythaemia.

29. D/D
• Neuroblastoma
• Hydronephrosis
• Renal cyst
• Renal cell carcinoma
• Lymphoma

30. Diagnosis
• Suspect in Abdominal masses
• Urine—haematuria.
• USG – indicate mass is intrarenal
• CT with contrast - Calcification seen in 5-10%
of cases.
• CXR – 10-20% have Pulmonary metastasis at
time of diagnosis.

31. Treatment
• Unilateral—nephrectomy – evaluate the other
kidney and liver.
• Followed by Chemo-
Vincristine + Actinomycin + Daunorubicin
For advanced cases other drugs-
Cisplatin, Carboplatin
• In advanced cases—Add Radiotherapy.

32. • Inoperable lesion- chemotherapy and
radiotherapy followed by nephrectomy later
• Bilateral tumor- nephrectomy on worse side
with radiotherapy to smaller tumor
• Stage IV – Pulmonary Irradiation + 3 drug
Chemo.

33. Prognosis
• Better- stage I
Age < 2 yrs
Tumor Wt <250gm
• Poor prognosis-
Recurrence of tumor

34. Stage 2 yr 5 yr
I 98% 97%
II 96% 94%
III 91% 88
IV 88% 82%
Anaplastic(III, 56% 54%
IV)

35. Thank you
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