neuroblastoma

Neuroblastoma,
CT of the abdomen
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Congenital
tumors: imaging when life just begins. Neuroblastoma. Case 1. Typical neuroblastoma. The abdominal ultrasound (a) and the axial fat-saturated T2-weighted images (b) show the well-defined, homogeneous mass arising from the right adrenal gland (white arrow) and displacing the kidney in this 2-day-old baby. Case 2. Axial contrast-enhanced CT (CECT) (c) shows the enhancing retroperitoneal tumor (white arrow) originating from the organ of Zückerkandl in this 10-day-old girl. Note the hepatic infiltration (arrow). Case 3. Axial CECT (d) shows the mass arising from the right paraspinal sympathetic chain and extending into the spinal canal in this 3-day-old baby boy. Note the enlargement of the spinal canal (white block arrow). Case 4. Atypical neuroblastoma. Axial ultrasound (e) and T1-weighted images after i.v. contrast medium administration (f) show the retroperitoneal, lobulated mass infiltrating the right kidney and causing hydronephrosis (arrow) in this 5-week-old baby. The lesion was initially considered to be a renal tumor, but histology revealed neuroblastoma
insightsimaging.springeropen.com • CC-by-2.0
Newborn with
a left abdominal mass and palpable subcutaneous nodules on the right chest wall, left thigh, and right buttock. Coronal (above left), sagittal (above right) and axial (below left) CT with contrast of the abdomen show a large left-sided suprarenal mass which is calcified and displaces the right kidney inferiorly and posterior. Axial CT of the pelvis (below right) shows the small right gluteal subcutaneous soft tissue nodule.The diagnosis was neuroblastoma Stage 4S.
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Newborn with
congenital heart disease. Coronal (above left) and sagittal (above right) CT with contrast of the chest obtained on the first day of life show an oval low density lesion superior to the right kidney. Coronal (below left) and sagittal (below right) CT with contrast of the abdomen obtained 5 weeks later show an increase in the size of the lesion superior to the right kidney. The diagnosis was neuroblastoma.
pediatricimaging.org • CC-by-nc-sa 4.0
Preschooler
with a left sided abdominal mass. AXR (above left) shows a distended stomach. Axial (above right), coronal (below left) and sagittal (below right) CT with contrast of the abdomen shows a large calcified left adrenal mass that is compressing and displacing the left kidney inferiorly and laterally.The diagnosis was neuroblastoma.
pediatricimaging.org • CC-by-nc-sa 4.0
Preschooler
with diffuse bone pain. Coronal (left) and axial (above right) CT with contrast of the abdomen show a large low density faintly calcified mass arising from the right adrenal gland. Axial image of the pelvis in bone windows (below) shows multiple lytic bone lesions throughout the iliac wings and sacrum.The diagnosis was neuroblastoma with diffuse bone metastases.
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Toddler with
opsoclonus and myoclonus. Axial CT with contrast of the pelvis (above) shows (from top to bottom in the midline) contrast in the base of the bladder, contrast in the rectum which is deviated to the right, and a solid soft tissue mass anterior to the sacrum. Axial (below left) and sagittal (below right) T2 MRI without contrast of the pelvis shows the presacral mass to have high signal intensity.The diagnosis was presacral neuroblastoma.
pediatricimaging.org • CC-by-nc-sa 4.0
Toddler with
a left-sided abdominal mass. Sagittal US of the left kidney (above left) shows a large, solid, hyperechoic mass superior to the left kidney. Axial CT with contrast of the abdomen (above right) shows an ill-defined solid mass in the region of the left adrenal gland, which (below right) crosses the midline and encases the mesenteric vasculature. Axial CT without contrast of the brain (below left) shows a lytic lesion in the right mandible.The diagnosis was neuroblastoma arising from the left adrenal gland with metastases to the skull.
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Neuroblastoma,
CT of the abdomen
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Teenager
status post radiation therapy to the spine now with a curvature to the spine. CXR AP (left) taken as an infant shows a round left-sided soft tissue mass that was in the posterior mediastinum and found to be neuroblastoma that was subsequently treated in part with radiation therapy. CXR AP (right) taken 17 years later shows a curvature of the upper thoracic spine convex to the right.The diagnosis was post radiation scoliosis in a patient with neuroblastoma.
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Neuroblastoma,
CT of the abdomen
Wikipedia • CC BY-SA 3.0
Infant with
an abdominal massSagittal T2 MRI shows a huge left adrenal mass that was noted to cross midline and encase the abdominal vasculature.The diagnosis was neuroblastoma.
pediatricimaging.org • CC-by-nc-sa 4.0
Infant with
recurrent pneumoniasCXR shows a posterior mediastinal mass to the left of the spine that is clearly calcified on the coronal and axial CT with contrast of the chest.The diagnosis was neuroblastoma.
pediatricimaging.org • CC-by-nc-sa 4.0
Toddler with
back painCoronal T1 MRI without contrast of the chest shows a right paraspinal mass in the region of the right adrenal gland that on axial sequences was seen to extend into the spinal canal.The diagnosis was neuroblastoma.
pediatricimaging.org • CC-by-nc-sa 4.0
Preschooler
with bilateral hip painAP radiograph of the femurs shows a mottled appearance to both femurs with multiple lytic lesions noted in the femurs with associated periosteal reaction, more prominent on the left than the right.The diagnosis was metastases from neuroblastoma.
pediatricimaging.org • CC-by-nc-sa 4.0
Infant with a
right orbital mass. Axial CT without contrast of the abdomen (above) shows a large mass that has faint calcifications within it that fills the entire left upper quadrant of the abdomen. Axial CT without (lower left) and with (lower right) contrast of the orbit shows a round soft tissue mass around the right pterygoid plate that enhances strongly with contrast.The diagnosis was neuroblastoma of the right adrenal gland with metastasis to the right orbit.
pediatricimaging.org • CC-by-nc-sa 4.0
Toddler with
constipation and urinary retention. Lateral image from a barium enema performed after an intravenous pyelogram (below) shows anterior displacement and compression of the bladder (to the left of the image) and rectum (in the middle of the image) by a presacral mass. The scout image (above) showed the mass was not calcified.The diagnosis was presacral neuroblastoma.
pediatricimaging.org • CC-by-nc-sa 4.0
School ager
status post radiation therapy to the spine for a right adrenal neuroblastoma now with a curvature to the spine. CXR AP shows a mild curvature of the thoracolumbar spine convex to the left. Several calcifications are seen projecting over the liver.The diagnosis was post radiation scoliosis in a patient with neuroblastoma.
pediatricimaging.org • CC-by-nc-sa 4.0
Infant with a
newly diagnosed adrenal mass. AP radiographs of the humerus (above) and radius and ulna (below) show lesions involving the proximal humeral diaphysis, entire radial diaphysis and proximal ulna diaphysis all of which are lytic in appearance and having a wide zone of transition and associated faint periosteal reaction.The diagnosis was bone metastases from neuroblastoma.
pediatricimaging.org • CC-by-nc-sa 4.0
Metastatic
involvement of the skull in neuroblastoma : a case report. Post contrast CT, axial view: a large heterogeneously enhancing soft-tissue adrenal mass in the right side abdomen; this right adrenal mass lesion is predominantly of low attenuation, which would suggest tissue necrosis.
www.eurorad.org • CC by-nc-sa-4.0
Metastatic
involvement of the skull in neuroblastoma : a case report. Sagittal reformation: a large heterogeneously enhancing soft-tissue adrenal mass in the right side abdomen. The right kidney is displaced inferiorly.
www.eurorad.org • CC by-nc-sa-4.0
A diagnostic
approach to the mediastinal masses. Neuroblastoma in a 20-year-old man. Axial (a) and coronal (b) T2-weighted MR images, and sagittal (c) contrast-enhanced T1-weighted MR image demonstrate an expansive and heterogeneous mass in the left paravertebral space which shows cystic degeneration within (*) as well as an intensive enhancement (in c). Note the spinal involvement (arrowhead in b)
insightsimaging.springeropen.com • CC-by-2.0
Toddler with
an abdominal mass. Axial CT with contrast of the abdomen shows a large non-calcified low density abdominal mass arising from the right adrenal gland which crosses the midline (above) and displaces the right kidney laterally (middle) and inferiorly (below).The diagnosis was neuroblastoma arising from the right adrenal gland.
pediatricimaging.org • CC-by-nc-sa 4.0
Radiopaedia • CC-by-nc-sa 3.0 • de

Neuroblastomas are tumors of neuroblastic origin. Although they may occur anywhere along the sympathetic chain, the vast majority arise from the adrenal gland.

They represent the most common extracranial solid childhood malignancy and are the third commonest childhood tumor after leukemia and brain malignancies. They account for ~15% of childhood cancer deaths.

Epidemiology

The tumors 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) .

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:

Pathology

The tumors arise from the primitive neuroectodermal cells or neural crest cells (adrenal medulla precursor). The histology is similar to small round blue cell tumors . The majority of them demonstrate chromosome 1p deletion and N-myc amplification.

Macroscopically, they tend to be large grey-tan colored soft lesions, with or without fibrous pseudocapsule; hence, some are well defined, and some are infiltrative. Areas of necrosis, hemorrhage, and particularly calcification, are very common.

Microscopically, they form Homer Wright rosettes . Most of them secrete catecholamines: vanillylmandelic acid (VMA) and homovanillic acid (HVA) .

Location

Neuroblastomas arise from the sympathetic nervous system :

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

Associations

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

Radiographic features

Plain radiograph

Appearances are non-specific, typically demonstrating an intrathoracic soft-tissue mass or an intra-abdominal 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.

Skeletal metastases are usually ill-defined and lucent, with periosteal reaction or metaphyseal lucency. Sclerotic metastases are uncommon .

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 .

CT

On CT, the tumor typically is heterogeneous with calcifications seen in 80-90% of cases . Areas of necrosis are of low attenuation.

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

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 .

  • T1: heterogeneous and iso to hypointense
  • T2
    • heterogeneous and hyperintense
    • cystic/necrotic areas very high intensity
    • signal voids may be evident
  • C+ (Gd): variable and heterogeneous enhancement
Nuclear medicine

Some compounds are used for diagnosis and staging:

  • pentetreotide labeled to Indium-In (a somatostatin analog)
    • not specific for neuroblastic tissue
  • MIBG (metaiodobenzylguanidine labeled to Iodine-)
  • FDG PET/CT

Surveillance for metastatic recurrence:

  • Tc-m MDP
    • 36% of primary tumors 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
    • diffuse infiltration (more common in stage 4S)
    • focal hypo-enhancing masses
  • lung and pleura
    • discrete nodules
    • diffuse consolidation
    • pleural disease is uncommon
  • brain and meninges
    • dural metastases can be diffuse or nodular
    • brain metastases are uncommon but variable in appearance

Treatment and prognosis

Treatment depends on the patient's stage. Localized tumors considered to be 'low-risk' are surgically excised, and patients tend to do very well (see below). In 'high-risk' tumors, a combination of surgery, chemotherapy +/- bone marrow transplantation is employed, unfortunately with poor overall results. In some cases, where tumors are very large, pre-surgical chemotherapy to attempt to downstage the tumor may be administered .

Patients with stage 1, 2, or 4S have a better prognosis. Unfortunately 40-60% of patients present with stage 3 or 4 diseases . For advanced disease, the age of the child is most important .

  • stage 1, 2 or 4S: 75-90% 3 year survival
  • stage 3
    • <1 year of age: 80-90% 1-year event-free survival
    • >1 year of age: 50% 3-year survival
  • stage 4
    • <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 the presence of metastasis
  • N-Myc mutation
  • chromosome 1p deletion
  • unfavorable Shimada histology index
Better prognostic factors
  • TRK-A expression

Differential diagnosis

For intrathoracic neuroblastoma consider:

For intra-abdominal neuroblastoma consider:

Assoziationen und Differentialdiagnosen zu Neuroblastom:
Rhabdomyosarkom
 
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Lungensequester
 
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Nephroblastom
 
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