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.
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
Clinical Features• Infants: - More localized –in cervical or intrathoracic areas.• Older : - Abdomen with disseminated disease.
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
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
Confirm• Tissue biopsy• Bone marrow aspiration• urine & S. catecholamine• DNA content- mycn amplification
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
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
Treatment• Surgical excision• Chemotherapy—• Cisplatin, Doxorubicin, Vincristine, cyclophos phamide—may help to convert unresectable tumour into a resectable one• Radiotherapy
Prognosis—3 year survival 95% 25-50% < 25%Age < 1yr > 1yr 1-5 yrsINSS 1,2,4s 3,4 3,4mycn N N Amplified
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
Intermediate risk: -• Intermediate risk: - stage – III – Chemo+Surgery+ Radiation stage IV < 1 yr - Surgery followed by Chemo• High risk —IV > 1yr —Chemo+ Surgery+ BMT
Metastasis• Local invasion- most common.invades surrounding tissue• Lymphatics – regional lymph nodes• Hematogenous – bone marrow,skeleton and liver• Rare- brain,lungs
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.
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
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.
STAGING – National W T Study• Stage IV Haematological metastasis—often to the lung.• Stage V Bilateral renal disease
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.
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.
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.
• 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.
Prognosis• Better- stage I Age < 2 yrs Tumor Wt <250gm• Poor prognosis- Recurrence of tumor