Neuroblastoma and Nephroblastoma

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Neuroblastoma and Nephroblastoma

  1. 1. Neuroblastoma and Nephroblastoma Dr. Kalpana Malla MD Pediatrics Manipal Teaching HospitalDownload more documents and slide shows on The Medical Post [ www.themedicalpost.net ]
  2. 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. 3. NEUROBLASTOMA (NB)• Variable clinical presentation.• May undergo spontaneous regressionTumour secretes –1. catecholamines- VMA,HVA2. neurone specificenolase(NSE)3 ferritin
  4. 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. 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. 6. Clinical Features• Infants: - More localized –in cervical or intrathoracic areas.• Older : - Abdomen with disseminated disease.
  7. 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. 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. 9. Clinical Features• Opsoclonus-Myoclonus: " dancing eyes, dancing feet syndrome"—better prognosis 4%• Cervical involvement: - Horners syndromeThorax:- Respiratory distress• Abdomen: - Hard mass, fixed, abdominal Discomfort, Massive hepatomegaly
  10. 10. Clinical Features• Vasoactive intestinal peptides - diarrhoea, hypokalemia• increased catecholamines - flushing, tachycardia, inc sweating,hypertension• Metastatic: - BM, bone, liver and skin.
  11. 11. Diagnosis:• Hemoblobin ↓• Platelets↓• LDH • NSE • Urea, creatinine • Inc. HVA, VMA.
  12. 12. Imaging• CXR• USG• CT• MRI• Bone scan
  13. 13. Confirm• Tissue biopsy• Bone marrow aspiration•  urine & S. catecholamine• DNA content- mycn amplification
  14. 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. 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. 16. Treatment• Surgical excision• Chemotherapy—• Cisplatin, Doxorubicin, Vincristine, cyclophos phamide—may help to convert unresectable tumour into a resectable one• Radiotherapy
  17. 17. Prognosis—3 year survival 95% 25-50% < 25%Age < 1yr > 1yr 1-5 yrsINSS 1,2,4s 3,4 3,4mycn N N Amplified
  18. 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. 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. 20. Metastasis• Local invasion- most common.invades surrounding tissue• Lymphatics – regional lymph nodes• Hematogenous – bone marrow,skeleton and liver• Rare- brain,lungs
  21. 21. WILMS 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.
  22. 22. 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
  23. 23. STAGING – National W T StudyStage 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.
  24. 24. STAGING – National W T Study• Stage IV Haematological metastasis—often to the lung.• Stage V Bilateral renal disease
  25. 25. 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.
  26. 26. Syndromes• WAGR – WT, Aniridia, Genitourinary malformations, Mental Retardation. Chr deletion 11 p 13• Beckwith Wiedemann – Organomegaly, Macroglossia, omphalocoele, he mihypertrophy. Chr deletion-11p15
  27. 27. Syndromes• Denys Drasch— WT, Nephropathy, Genital abnormalities.• Familial WT• Perlman syndrome• Paraneoplastic syndrome- Inc Erythropoeitin with Polycythaemia.
  28. 28. D/D• Neuroblastoma• Hydronephrosis• Renal cyst• Renal cell carcinoma• Lymphoma
  29. 29. 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.
  30. 30. Treatment• Unilateral—nephrectomy – evaluate the other kidney and liver.• Followed by Chemo- Vincristine + Actinomycin + DaunorubicinFor advanced cases other drugs- Cisplatin, Carboplatin• In advanced cases—Add Radiotherapy.
  31. 31. • 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.
  32. 32. Prognosis• Better- stage I Age < 2 yrs Tumor Wt <250gm• Poor prognosis- Recurrence of tumor
  33. 33. Stage 2 yr 5 yrI 98% 97%II 96% 94%III 91% 88IV 88% 82%Anaplastic(III, 56% 54%IV)
  34. 34. Thank youDownload more documents and slide shows on The Medical Post [ www.themedicalpost.net ]

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