This document discusses neuroblastoma and nephroblastoma. [1] Neuroblastoma is the most common extracranial tumor in children and accounts for 8% of childhood cancers. It commonly presents before age 5 and can secrete catecholamines. [2] Nephroblastoma, also known as Wilm's tumor, is the most common renal tumor in children. It typically presents before age 3 as an abdominal mass. Staging and treatment involve surgery with or without chemotherapy and radiation depending on risk factors. Both tumors can metastasize but have better prognosis when caught early.

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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