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Nephroblastoma
Dr. Kalpana Malla
MD Pediatrics
Manipal Teaching Hospital
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Nephroblastoma
Dr. Kalpana Malla
MD Pediatrics
Manipal Teaching Hospital
Download more documents and slide shows on The Medical Post [ www.themedicalpost.net ]](https://image.slidesharecdn.com/newborn-neuroandnephroblastoma-120107084301-phpapp02/75/Neuroblastoma-and-Nephroblastoma-1-2048.jpg)
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Neuroblastoma and Nephroblastoma
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.
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Neuroblastoma and Nephroblastoma
- 1. Neuroblastoma and Nephroblastoma Dr. Kalpana Malla MD Pediatrics Manipal Teaching Hospital Download more documents and slide shows on The Medical Post [ www.themedicalpost.net ]
- 2. NEUROBLASTOMA (NB) • Mostcommon 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) • Variableclinical 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 • Anysite 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 • Appearschronically 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 • Vasoactiveintestinal 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 • StageI- 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 • StageIII- 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: - • Favourablehistology - 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 – NationalW 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 – NationalW 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 inAbdominal 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)
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