neuroblastoma and nephroblastoma

Neuroblastoma and Nephroblastoma

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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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