DEFINITION-

1) Malignant tumor of primitive neural crest cell.

2) Most common extra cranial solid malignancy in children.

3) 3 rd common pediatric malignancy.

General features –

Diagnostic clue- suprarenal; mass, with calcification.

 

Location –

1) Adrenal medulla (35%)

2) Extra adrenal retroperitoneum (35%)

3) Posterior mediastinum (20%)

4) Neck and pelvis (2-3%)

5) Mets with no primary identified. (1%).

 

General imaging features-

1) Mostly arises from adrenal medulla, but can arise any where from sympathetic chain from neck to pelvis.

2) Aggressive tumor, tendency to invade adjacent str, surrounds and engulf vascular str, rather than displaces it, like SMA, aorta, celiac artery.

3) Tendency to invade to neuroforamina,

4) Metastasis to bone and liver.

 

Prognosis-

1) <1years – better prognosis- metastasis to liver and skin

2)>1years  - bad prognosis – metastasis to bone .

 

Genetics and associate abnormality-

1) n-myc proto oncogene a/w poor prognosis.

2) CD44 glycoprotein on surface A/W better prognosis.

3) It is associated with Von Reckling Hausen disease, Beckwith wiedemann syndrome, hirshprunge disease.

 

 Clinical features-

1) First symptoms of neuroblastoma are often vague and may include fatigue and loss of appetite.

2) the abdomen, a tumor may cause a swollen belly and constipation.

3) tumor in the chest may cause breathing problems.

4) Tumors pressing on the spinal cord cause a feeling of weakness.

5) tumor in the head may cause the eyes to start to swell outwards and turn black due to the pressure from behind.

 

Staging –

                    International Neuroblastoma Staging System" (INSS)

1) Stage 1: Localized tumor confined to the area of origin.

2) Stage 2A: Unilateral tumor with incomplete gross resection; identifiable ispilateral and contra lateral lymph node negative for tumor.

3) Stage 2B: Unilateral tumor with complete or incomplete gross resection; with ispilateral lymph node positive for tumor; identifiable contra lateral lymph node negative for tumor.

4) Stage 3: Tumor infiltrating across midline with or without regional lymph node involvement; or unilateral tumor with contralateral lymph node involvement; or midline tumor with bilateral lymph node involvement.

5) Stage 4: Dissemination of tumor to distant lymph nodes, bone marrow, bone, liver, or other organs except as defined by Stage 4S.

6) Stage 4S: Age <1 y.o. with localized primary tumor as defined in Stage 1 or 2, with dissemination limited to liver, skin, or bone marrow (less than 10 percent of nucleated bone marrow cells are tumors).

 

Radiographic finding- 

Radiography-

1)Non specific soft tissue mass, calcification 30%.

2)widening of inferior thoracic Para spinal soft tissue may be only radiologic finding of upper abdominal mass.

3)Bone mets –lucent sclerotic mixed.

CT finding –

1)heterogenous mass area of necrosis and hemorrhage .

2) calcification seen in 85%

3)invasive pattern of growth, engulf vessels than displacing it.

MRI finding –

1) High signal on T2 weighted images,and low signal on T1 weighted images.

2) heterogenous signal in relation to calcification , hemorrhage , necrosis.

1) Increased echogenicity , heterogenous.

2)calcification causes shadowing echogenicities.

3)varying echogenicity with necrosis and hemorrhage.

4) increased vascularity on colour Doppler.

5) suprarenal than arising from kidney.

1)Bone scan –

a) Technetium 99m MDP.

b) uptake seen in bony metastasis .

c)calcified primary mass demonstrated in 74% cases .

2) PET scan – role of PET not defined in neuroblastoma.

3)MBG-

a) Metaiodobenzylguanidine.

b) avid uptake in relation catecholamine production.

c) approximately 30% are not MIBG avid.

 

1)Wilms tumor- 3 years, calcification uncommon.

                           Grow like ball, displaces vessels, arising from kidney.

2)Neonatal adrenal hemorrhage .

3)Pheochomocytoma

4) Adrenal carcinoma.