Neuroblastoma and Nephroblastoma Download more documents and slide shows on The Medical Post [ www.themedicalpost.net ] Dr. Kalpana Malla MD Pediatrics Manipal Teaching Hospital
May 11, 2015
Neuroblastoma and Nephroblastoma
Download more documents and slide shows on The Medical Post [ www.themedicalpost.net ]
Dr. Kalpana MallaMD Pediatrics
Manipal Teaching Hospital
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
NEUROBLASTOMA (NB)
• Variable clinical presentation.• May undergo spontaneous regressionTumour secretes –1. catecholamines- VMA,HVA2. neurone specificenolase(NSE)3 ferritin
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
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
Clinical Features
• Vasoactive intestinal peptides - diarrhoea, hypokalemia
• increased catecholamines - flushing, tachycardia, inc sweating,hypertension
• Metastatic: - BM, bone, liver and skin.
Diagnosis:
• Hemoblobin ↓• Platelets↓• LDH • NSE • Urea, creatinine • Inc. HVA, VMA.
Imaging
• CXR• USG• CT• MRI• Bone scan
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,
cyclophosphamide—may help to convert unresectable tumour into a resectable one
• Radiotherapy
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
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
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.
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 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.
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.
Syndromes
• WAGR – WT, Aniridia, Genitourinary malformations,
Mental Retardation. Chr deletion 11 p 13• Beckwith Wiedemann – Organomegaly, Macroglossia,
omphalocoele, hemihypertrophy. Chr deletion-11p15
Syndromes
• Deny's Drasch— WT, Nephropathy, Genital abnormalities.• Familial WT• Perlman syndrome• Paraneoplastic syndrome- Inc Erythropoeitin with Polycythaemia.
D/D
• Neuroblastoma • Hydronephrosis• Renal cyst• Renal cell carcinoma• Lymphoma
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 + Daunorubicin For 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
Stage 2 yr 5 yr
I 98% 97%
II 96% 94%
III 91% 88
IV 88% 82%
Anaplastic(III, IV)
56% 54%
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