Neuroblastoma pediatric

Clinical presentation• The usual finding in a child with NBL is an abdominal mass; additional findings are renin-associated hypertension from renal artery compression • Unwell from met

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

age of 2–3 years

• Boys are more frequently affected than girls.

Sites of origin

• NBLs arise from the adrenal glands or follow the distribution of the sympathetic ganglia along paraspinal areas from the neck to the pelvis

• The most common primary site for NBL

development is the retroperitoneum, the

adrenal medulla (35%) and the extra-adrenal paraspinal ganglia (30%–35%)

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. 

Clinical presentation

• The usual finding in a child with NBL is an abdominal mass; additional findings are renin-associated

hypertension (from renal artery compression)

• Unwell from metastatic spread of the tumour:

Generalised skeletal pain or even arthritis-type

complaints, effects of hormone production and specific findings from bone marrow involvement,

non-such as weight loss, malaise, anaemia, fever and

irritability, can be encountered

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

• On Ultrasound the tumor is generally

echogenic and inhomogeneous with bright

calcifications.

A feature of neuroblastoma is the tendency of vascular encasement and they have a

tendency to grow between the vertebral

column and the aorta which is lifted ventrally.

• 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. 

• 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 

• T2

– 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

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 left-sided NBL mass with bone  

marrow involvement (blue arrow).

Neuroblastoma vs Wilms tumor

Neuroblastoma

• calcification very common: 90%

• encases vascular structures but does not

• 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

• NBLs have a variable prognosis; tumour stage,

patient age, tumour oncogenes and DNA content

are all known to be implicated. 

• Prognosis is highly variable at different ages Some behave aggressively while others, typically in

infancy, may spontaneously regress Low to

intermediate risk tumours tend to have a reasonably good prognosis (90% survival approximately) with high-risk tumours being much less favourable (40-

50% survival)

• The MYCN oncogene is responsible for providing the

code used by proteins in tissue development If this

mutates, which can be signalled by abnormal

amplification, cancerous cells can develop and the

resulting mass is more resistant to treatment, thus it has

a more unfavourable outcome

• Tumours with MYCN amplification, whether localised or metastatic, are all categorised as high risk tumours in

both North American Children's Oncology Group (COG) and European (SIOPEN) neuroblastoma studies. 

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 

• Chromosome 11q 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-childhoo d/stages-and-groups

radiologyassistant.nl/pediatrics/abdominal-masses/ma lignant-abdominal-tumors-in-children

cancerimagingjournal.biomedcentral.com/articles/10 1186/s40644-015-0040-6