Neuroblastoma pediatric

a Abdominal US, sagittal plane, shows a heterogeneous lesion with multiple internal echogenic foci in the right retroperitoneum that displaces the diaphragm superiorly.. • On CT, NB

Neuroblastoma pediatric

• Neuroblastoma (NBL) along with ganglioneuroblastoma and ganglioneuroma constitute a group of tumours of ganglion cell origin that derive from primordial neural crest cells, which are the precursors of the sympathetic nervous system

• The degree of malignancy is designated by the degree of cellular and

extra-cellular maturation of these tumours The most undifferentiated and aggressive NBL presents in young children (median age 2 years).    

• The median age at diagnosis is 22 months More than 90% of the diagnosed cases are children aged 5 years, with peak incidence at age of 2–3 years  

• Boys are more frequently affected than girls

Biologic prognostic factors

• the Myc-N gene, a proto-oncogene located on the distal end of the chromosome

arm 2p, was found to be amplified, present in multiple (>10) copies, in 20%–30%

of NBLs. Myc-N amplification is associated with rapidly progressive disease and a

poor outcome

Clinical presentation

• NBLs can be discovered incidentally

• Pediatric abdominal tumors are often very large at initial presentation, because most children come to attention because someone noted severe abdominal distention. 

• It may seem a contradiction, but in very large tumors, it is usually more difficult

to ascertain the organ of origin. 

Plain radiograph

• Appearances are non-specific, typically

demonstrating an intra-abdominal

soft-tissue mass displacing adjacent organs

Pressure on adjacent bones may cause

remodeling of ribs, vertebral bodies or

pedicle thinning Up to 30% may have

evidence of calcification on the plain film.

• 3.5-year-old girl with malaise (a)

Abdominal US, sagittal plane, shows a

heterogeneous lesion with multiple

internal echogenic foci in the right

retroperitoneum that displaces the

diaphragm superiorly (b) Abdominal US,

axial plane at the level of the renal

arteries, shows anterior displacement of

the aorta, IVC and renal arteries with

preserved patency. 

• On CT, NBLs present as large, heterogeneous, lobulated soft-tissue masses that show heterogeneous or little enhancement

• Coarse, finely stippled or curvilinear calcifications are seen in 85% of the

abdominal NBLs on CT Low attenuation areas seen within the tumour represent pseudo-necrosis or haemorrhage

• Adjacent organs are usually displaced, although in more aggressive tumors

direct invasion of the psoas muscle   or kidney can be seen The latter can make distinguishing neuroblastoma from Wilms tumor difficult. 

• Lymph node enlargement is often present. 

• pre-operative surgical planning

• contrast-enhanced images can delineate the vasculature to best effect

• post-chemotherapy, the solid portions of the mass are easier to define than on MRI and the extent of calcification, which increases after treatment and which can be important for the surgeon to appreciate before surgery, is more easily characterised

Large heterogenously enhancing mass compressing the left kidney.

Cavernous transformation of the portal vein likely secondary to obstruction

by mass.

Ascites.

Imaging of a 2.5-year-old girl with malaise and abdominal distension (a) Coronal CT reconstruction after injection of contrast medium shows a large heterogeneous low attenuating lesion with calcification in the right suprarenal area It extends across the midline, and surrounds the IVC Secondary involvement of the liver is also noted (b) Coronal CT reconstruction in the same patient post chemotherapy, shows significant reduction in the size of the primary lesion but the portal vein remains encased (inoperable NBL).

• MRI should now be the cornerstone imaging modality for all primary NBL

tumours whether in the neck, chest, abdomen or pelvis

• MRI can easily assess the extent of disease, being superior to CT in assessing metastatic marrow disease, chest wall invasion and spinal canal involvement

• Epidural extension of NBL and leptomeningeal dissemination are better assessed with MRI which should be performed on any child with paraspinal NBL

• On MRI, the tumour is typically heterogeneous with a variable enhancement pattern

• T1: heterogeneous and iso to hypointense 

– heterogeneous and hyperintense

– cystic/necrotic areas very high intensity

• C+ (Gd): variable and heterogeneous enhancement 

• Cystic and haemorrhagic areas within the tumour can be convincingly identified but not calcification

• On diffusion-weighted images, NBLs show increased tumour signal which is

attributed to restricted diffusion

• Bone marrow disease is usually seen as diffuse infiltration but it may also

present a nodular pattern with areas of low and high signal intensity on T1W and T2W images, respectively

Click to edit Master text styles

Second level

Third level

Fourth level

Fifth level

Coronal T2 MR of a 3 year old boy with extensive abdominal NBL that crosses  

the midline and is here seen to encase the aorta (blue arrow).

Axial T2 MR of 2 year old girl showing NBL with rib invasion (blue   arrow), anterior aortic displacement and encasement (red arrow) and

bilateral pleural effusions.

Axial T2 MR of 3 year old boy showing intraspinal extent of NBL with tumour  

seen in both neural foramina on this single image (blue arrows).

Coronal T2 MR of a 2 year old boy showing lef-sided NBL mass with   bone marrow involvement (blue arrow).

Neuroblastoma vs Wilms tumor

Neuroblastoma

• calcification very common: 90%

• encases vascular structures but does not invade

• more commonly crosses the midline, especially

behind the aorta  

• more common to have extension into the chest

• bone metastases are common (

Hutchinson syndrome)

• extension into spinal canal can be seen

• retroperitoneal lymph nodes are more often

• slightly older age group: peak 3-4 years of age

• well-circumscribed

• claw sign with the kidney  

• extension into IVC/renal vein

• bone metastases are rare, rather lung metastases are common

• extension into spinal canal never seen

• retroperitoneal lymphadenopathy is uncommon

• higher incidence of hemorrhage

The International Neuroblastoma Risk Group (INRG)

Preliminary evidence suggests absence of IDRFs leads to more complete resection, with the presence of IDRFs resulting in more

post-operative morbidity 

Using the following factors, each neuroblastoma is classified into 1 of 4 categories: very low-risk, low-risk, intermediate-risk, or high-risk.

• The stage of the disease according to the INRG staging system

• The child's age at the time of diagnosis

• Histologic category, such as maturing ganglioneuroma versus

ganglioneuroblastoma, intermixed versus ganglioneuroblastoma, or nodular versus neuroblastoma

• Grade, or how cells of the tumor are differentiated

• MYCN gene status 

• Tumor cell ploidy, which is the DNA content of tumor cells

• Treatment is tailored according to the risk assignment Most patients with very low-risk and low-risk disease commonly receive surgery alone Sometimes, infants with a small localized tumor have been successfully watched closely without any surgery, an approach called "observation" or "wait and watch."

• Patients with intermediate-risk disease receive surgery and chemotherapy

• A very intensive approach, often using several types of treatments, is used for patients with high-risk neuroblastoma

• https://

www.cancer.net/cancer-types/neuroblastoma-childhood/stages-and-groups

• https://

radiologyassistant.nl/pediatrics/abdominal-masses/malignant-abdominal-tumors-in-children

• https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1665241/

• https://radiopaedia.org/articles/neuroblastoma-vs-wilms-tumour-1?lang=us

• https://

cancerimagingjournal.biomedcentral.com/articles/10.1186/s40644-015-0040-6