Neuroblastoma and Nephroblastoma

1. Neuroblastoma and Nephroblastoma Download more documents and slide shows on The Medical Post [ www.themedicalpost.net ] Dr. Kalpana Malla MD Pediatrics Manipal Teaching Hospital

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, cyclophosphamide—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

21. Nephroblastoma

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, hemihypertrophy. 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, IV) 56% 54%

35. Thank you Download more documents and slide shows on The Medical Post [ www.themedicalpost.net ]