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    Neuroblastoma Radiopedia

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    roni pahlevi
    Neuroblastoma is the third most common childhood tumour. It typically occurs in infants and young children, presenting as a painless abdominal mass. Neuroblastomas arise from neural crest cells and can occur throughout the sympathetic nervous system, most commonly in the adrenal glands or retroperitoneum. Staging is based on extent of disease, with stage 4 indicating metastatic disease. Treatment involves surgery, chemotherapy, and sometimes bone marrow transplantation, with prognosis varying based on age, stage, and other risk factors.

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    Neuroblastoma Radiopedia

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    roni pahlevi
    177 views8 pages
    Neuroblastoma is the third most common childhood tumour. It typically occurs in infants and young children, presenting as a painless abdominal mass. Neuroblastomas arise from neural crest cells and can occur throughout the sympathetic nervous system, most commonly in the adrenal glands or retroperitoneum. Staging is based on extent of disease, with stage 4 indicating metastatic disease. Treatment involves surgery, chemotherapy, and sometimes bone marrow transplantation, with prognosis varying based on age, stage, and other risk factors.

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    pembagian jenis neuroblastoma

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    Neuroblastoma is the third most common childhood tumour. It typically occurs in infants and young children, presenting as a painless abdominal mass. Neuroblastomas arise from neural crest cells and can occur throughout the sympathetic nervous system, most commonly in the adrenal glands or retroperitoneum. Staging is based on extent of disease, with stage 4 indicating metastatic disease. Treatment involves surgery, chemotherapy, and sometimes bone marrow transplantation, with prognosis varying based on age, stage, and other risk factors.

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    Download as DOCX, PDF, TXT or read online from Scribd
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    177 views8 pages

    Neuroblastoma Radiopedia

    Uploaded by

    roni pahlevi
    Neuroblastoma is the third most common childhood tumour. It typically occurs in infants and young children, presenting as a painless abdominal mass. Neuroblastomas arise from neural crest cells and can occur throughout the sympathetic nervous system, most commonly in the adrenal glands or retroperitoneum. Staging is based on extent of disease, with stage 4 indicating metastatic disease. Treatment involves surgery, chemotherapy, and sometimes bone marrow transplantation, with prognosis varying based on age, stage, and other risk factors.

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    of 8

    Neuroblastoma

    Dr Amir Rezaee and Dr Elhamy Bekhit et al.


    Neuroblastomas are tumours of neuroblastic origin corresponding to the most common extracranial
    solid childhood malignancies, and the third commonest childhood tumours after leukaemia and brain
    malignancies. It accounts for ~15% of childhood cancer deaths 2.

    Epidemiology
    The tumours typically occur in infants and very young children (mean age of presentation being 22 months)
    with 95% of cases diagnosed before the age of 10 years. Occasionally, they may be identified antenatally or
    immediately at birth (see congenital neuroblastoma) 2.

    Clinical presentation
    Typically with pain or a palpable mass and abdominal distension, although numerous other presentations may
    be encountered due to local mass effect.
    Other accompanying syndromes include:

    Hutchinson syndrome: bony metastases may present with skeletal pain or a palpable lump or limping
    and irritability due to skeletal metastases

    Pepper syndrome: hepatomegaly due to extensive liver metastasis

    blueberry muffin syndrome: multiple cutaneous lesions

    opsomyoclonus 5: rapid, involuntary conjugate fast eye movements

    Pathology
    The tumours arise from the primitive neuroectodermal cells or neural crest cells (adrenal medulla precursor).
    The histology is similar to small round blue cell tumours 3. The majority of them demonstrate chromosome 1p
    deletion and N-myc amplification.
    Macroscopically, they tend to be large grey tan colour, soft lesions with or without fibrous pseudocapsule hence
    some are well defined and some are infiltrative. Areas of necrosis, haemorrhage, and particularly calcification
    are very common.
    Microscopically, they form Homer Wright rosettes 3. Most of them secrete catecholamines: vanillylmandelic acid
    (VMA) and homovanillic acid (HVA) 2.

    Location
    Neuroblastomas arise from the sympathetic nervous system 2-3:

    Intra-abdominal disease (two-thirds of cases) is more prevalent than the intrathoracic disease. Specific sites
    include:

    adrenal glands: most common site of origin, 35%

    retroperitoneum: 30-35%
    o

    organ of Zuckerkandl

    coeliac axis

    paravertebral sympathetic chain

    posterior mediastinum: 20%

    neck: 1-5%

    pelvis: 2-3%

    Associations
    The vast majority of neuroblastomas are sporadic, however in rare instances they may be associated with

    Beckwith-Wiedemann syndrome

    central failure of ventilation

    DiGeorge syndrome

    Hirschsprung disease

    neurofibromatosis type 1

    1-4

    Radiographic features
    Radiograph
    Appearances are non-specific, typically demonstrating an intrathoracic or intraabdominal soft-tissue mass.
    Pressure on adjacent bones may cause remodelling of ribs, vertebral bodies or pedicle thinning. Up to 30%
    may have evidence of calcification on the plain film.
    Skeletal metastases are usually ill-defined and lucent, with periosteal reaction or metaphyseal lucency.
    Sclerotic metastases are uncommon 2.

    Ultrasound
    Neuroblastoma on ultrasound demonstrates a heterogeneous mass with internal vascularity. Often there are
    areas of necrosis that appear as regions of low echogenicity. Calcification may or may not be evident on
    ultrasound 2.

    CT

    On CT, the tumour typically is heterogeneous with calcifications seen in 80-90% of cases 2. Areas of necrosis
    are of low attenuation.
    The tumour morphology is often helpful, with the mass seen insinuating itself beneath the aorta and lifting it off
    the vertebral column. It tends to encase vessels and may lead to compression. Adjacent organs are usually
    displaced, although in more aggressive tumours direct invasion of the psoas muscle or kidney can be seen. In
    the latter it can make distinguishing neuroblastoma from Wilms tumour difficult (see neuroblastoma vs. Wilms
    tumour).
    Lymph node enlargement is often present.

    MRI
    MRI is superior to all other modalities in assessing the organ of origin, intracranial or intraspinal disease and
    bone marrow disease 2.

    T1: heterogeneous and iso to hypointense

    T2
    o

    heterogeneous and hyperintense

    cystic/necrotic areas very high intensity

    signal voids may be evident

    C+ (Gd): variable and heterogeneous enhancement

    Nuclear medicine
    A number of compounds are used for diagnosis and staging:

    pentetreotide labeled to Indium-In111 (somatostatin analog)


    o

    not specific for neuroblastic tissue


    MIBG (metaiodobenzylguanidine labeled to Iodine-123)

    95% of neuroblastomas secrete catecholamines, however, 10-30% of neuroblastomas are


    negative on MIBG

    sensitivity: 88%

    specificity: 99% (for sympathetic tissue) 2

    does not distinguish between


    neuroblastoma, glanglioneuroblastoma, ganglioneuroma, carcinoid, andpheochromocytoma

    FDG PET/CT

    Surveillance for metastatic recurrence

    Tc-99m MDP
    o

    36% of primary tumours negative

    mainly to evaluate skeletal metastases

    also able to detect some lung and liver metastases

    Staging and metastatic disease


    For staging refer to: neuroblastoma staging
    Metastatic disease is common and has a variety of patterns:

    bone
    most common

    liver
    o

    diffuse infiltration (more common in stage 4S)

    focal hypo-enhancing masses

    lung and pleura


    o

    discrete nodules

    diffuse consolidation

    pleural disease is uncommon

    brain and meninges


    o

    dural metastases can be diffuse of nodular

    brain metastases are uncommon but variable in appearance

    Treatment and prognosis


    Treatment depends on the patients stage. Localised tumours considered to be 'low-risk' are surgically excised
    and patients tend to do very well (see below). In 'high-risk' tumours, a combination or surgery, chemotherapy
    +/- bone marrow transplantation is employed, unfortunately with poor overall results. In some cases, where
    tumours are very large, pre-surgical chemotherapy to attempt to down-stage the tumour may be administered 2.
    Patients with stage 1, 2 or 4S have a better prognosis. Unfortunately 40-60% of patients present with stage
    3 or 4 disease 4. For advanced disease, the age of the child is most important 3.

    stage 1, 2 or 4S: 75-90% 3 year survival

    stage 3
    o

    <1 year of age: 80-90% 1 year event free survival

    >1 year of age: 50% 3 year survival

    stage 4
    o

    <1 year of age: 60-75% 1 year event free survival

    >1 year of age: 15% 3 year survival

    Poor prognostic factors

    later age of onset: >18 months

    higher stage: particularly in presence of metastasis

    N-Myc mutation

    chromosome 1p deletion

    unfavourable Shimada histology index

    Better prognostic factors

    TRK-A expression

    Differential diagnosis
    For an intra-thoracic neuroblastoma consider:

    intrathoracic lymphoma

    extra lobar pulmonary sequestration

    round pneumonia

    ganglioneuroma

    ganglioneuroblastoma

    For an intra-abdominal neuroblastoma consider:

    ganglioneuroma

    ganglioneuroblastoma

    rhabdomyosarcoma

    Wilms tumour

    References
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    Article Information:

    Cases and Figures

    Case 2: retroperitoneal mass


    Case 3

    Case 4

    Case 5

    Case 6


    Case 7

    Case 9

    Case 10: thoracic

    Case 11


    Case 12: metastases

    Imaging Differential Diagnosis

    Wilms tumour

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