AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Differentials
• Radiograph
• CT Scan
• MRI
• Ultrasound
• Nuclear Medicine
• Intervention
• Multimedia
• References

INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Differentials
• Radiograph
• CT Scan
• MRI
• Ultrasound
• Nuclear Medicine
• Intervention
• Multimedia
• References

Background

Neuroblastoma is the most common extracranial pediatric neoplasm and the third most common pediatric malignancy after leukemia and CNS tumors. Neuroblastomas can arise from anywhere along the sympathetic chain. They have been associated with a number of disorders, such as Hirschsprung disease, fetal alcohol syndrome, DiGeorge syndrome, Von Recklinghausen disease, and Beckwith-Wiedemann syndrome.

Pathophysiology

Neuroblastomas arise from primitive neural crest cells that differentiate to form the sympathetic nervous system. They are related to ganglioneuroblastomas and ganglioneuromas. All 3 tumors arise from primitive neural crest cells, and they can be differentiated on the basis of the presence and percentage of immature, undifferentiated sympathetic cells or neuroblasts. Neuroblastomas consist predominantly of neuroblasts, where ganglioneuromas are composed entirely of well-differentiated cells. Ganglioneuroblastomas contain about 50% or more mature cells. Malignant potential is proportional to the percentage of immature cells in the tumor with neuroblastomas being the most malignant of the three and ganglioneuromas being benign.

Histologically, neuroblasts are small, round cells with dark nuclei and small indistinct nucleoli. They contain little cytoplasm. The cells grow in sheets and have ill-defined borders. Neuroblastomas often have Homer-Wright rosettes. These are circular patterns of neoplastic cells arranged around a core of neuropil (fibrillar extensions of neuroblasts). They are typical of neuroblastomas but are not present in all cases. Gross specimens of neuroblastomas can appear well circumscribed or infiltrative. They do not have capsules. They range from minute nodules or in situ lesions to large masses weighing more than 1 kg.

Neuroblastomas exhibit a great variety of tumor biologic behaviors that can be used to determine a patient's prognosis. About 95% of neuroblastomas secrete catecholamines (vanillylmandelic acid [VMA] and homovanillic acid [HVA]), though patients rarely have symptoms related to catecholamine secretion. HVA is a dopamine metabolite and is a more mature catecholamine than VMA, which is a metabolite of epinephrine and norepinephrine. Increased levels of HVA in the urine are correlated with maturity of the tumor and an improved prognosis.

Nearly 7% of neuroblastomas secrete vasoactive intestinal peptide (VIP). These tumors are more mature; therefore, patients with VIP-producing tumors have a prognosis better than that of other patients. Elevated levels of serum ferritin (>142 ng/mL) and neurospecific enolase (>100 ng/mL) are associated with a bad prognosis.

Certain genetic factors can also affect the prognosis. N-MYC is a proto-oncogene located on chromosome arm 2p. If it is present in multiple copies (10 or more), it promotes rapid tumor growth and indicates a bad prognosis. Deletion of the short arm of chromosome 1 causes rapid tumor growth due to a presumed loss of a tumor suppressor gene, indicating a bad prognosis. Cells with normal or near normal DNA content (DNA index = 1) are associated with aggressive tumor activity. Hyperdiploid cells (DNA index >1) are associated with a better prognosis since this DNA complement may stimulate the proliferation of Schwann cells and promote maturity.

Frequency

United States

Approximately 500-525 cases of neuroblastoma are diagnosed each year. It accounts for 8-10% of all pediatric malignancies. The incidence of neuroblastoma in the United States is 8.0-8.7 cases per million people. Neuroblastoma is the most common neonatal malignancy, accounting for 30-50% of all neoplastic cases in neonates.

International

In general, the incidence in other industrialized nations appears to be similar to that observed in the United States. The one exception is Japan. Japan has a higher incidence of neuroblastoma than anywhere else in the world. This was felt to be the result of neonatal screening for neuroblastoma, which presumably detected tumors that normally would have not been discovered and would have regressed spontaneously. Neonatal screening for neuroblastoma has been abandoned in Japan since it was shown not to significantly improve mortality or morbidity rates. Other neonatal screening studies in Germany and Quebec likewise showed no benefit in neonatal screening on mortality and morbidity rates (Woods, 2002; Schilling, 2002).

Mortality/Morbidity

Neuroblastomas account for 8-10% of all pediatric malignancies but they account for 15% of deaths from cancer in the pediatric population.

Race

No racial predilection is recognized.

Sex

The prevalence of neuroblastoma does not differ by sex.

Age

The median age at diagnosis is 22 months. Up to 95% of cases are diagnosed by the age of 10 years. Neuroblastomas have been diagnosed in utero as early as 19 weeks' gestational age.

Anatomy

Neuroblastomas can arise from anywhere along the sympathetic chain. They most commonly occur in the adrenal medulla (35%). Usually only 1 adrenal gland is involved, and bilateral involvement is rare. The adrenal medulla receives a significant amount of innervation from the sympathetic nervous system because the secretory cells of the adrenal gland are derived from neural crest cells.

Neuroblastomas also occur as primary tumors in the extra-adrenal retroperitoneum (from the sympathetic trunk, celiac ganglion, superior and inferior mesenteric ganglia) in 30% of cases, in the posterior mediastinum in 20% of cases (from the sympathetic trunk and the aortic body), in the neck in up to 5% of cases (carotid body), and in the pelvis in 5% of cases (from the organ of Zuckerkandl).

Primary intracranial neuroblastoma is rare, occurring in 2% of patients; this usually arises from the olfactory bulb. Neuroblastomas arising from the olfactory bulb are called esthesioneuroblastomas. Rare cases of primary neuroblastoma in the lung, thymus, stomach, kidney, or cauda equina have been documented. About 1% of patients present with evidence of metastatic disease but with no identifiable primary tumor. Common locations of neuroblastoma metastases are bone (60%), regional lymph nodes (45%), orbit (20%), liver (15%), intracranial areas (14%), and lung (10%).

Clinical Details

Clinical findings

Two thirds of patients with neuroblastoma present with metastases at the time of diagnosis. They often present with constitutional symptoms, such as weight loss, malaise, anorexia, anemia, and irritability. One third have fever.

Approximately 45-54% of patients with neuroblastoma have a palpable abdominal mass. These patients may have abdominal pain. Nearly 10% of patients develop hypertension as a result of renal vein compression. Hypertension in patients with neuroblastoma may also be related to renal arterial compression and excess catecholamine production. Extradural extension of neuroblastomas can present with focal or diffuse paralysis and bowel or bladder dysfunction. Pelvic neuroblastomas can also cause bowel or bladder dysfunction.

Bone metastases can cause focal pain, which can simulate osteomyelitis. Bone metastases are often metaphyseal and symmetrical. Persistent, diffuse or migratory pain can be confused with juvenile rheumatoid arthritis or leukemia. Patients with bone metastases often present with limping and irritability. This syndrome has been known as Hutchinson syndrome.

Some patients present with proptosis secondary to tumor invasion of the retrobulbar soft tissues. These patients may also present with periorbital ecchymosis (raccoon eyes), which can mimic child abuse. Cervical neuroblastomas can mimic cervical adenitis. Horner syndrome can occur in the presence of cervical and thoracic neuroblastomas. Myoclonic encephalopathy (opsomyoclonus and cerebellar ataxia) occurs in 2% of patients with neuroblastoma. About 50% of patients with this syndrome have neuroblastoma.

An intractable watery diarrhea occurs in up to 7% of patients secondary to secretion of VIP. This can mimic malabsorption.

Hepatic metastases are common and can be nodular or diffuse. Massive liver metastases can result in severe increased intra-abdominal pressure. This has been known as Pepper syndrome.

Skin metastases have also been documented. They may appear dark blue, resembling blueberries. The blueberry-muffin syndrome occurs when there are multiple skin metastases.

Fetal neuroblastoma can be detected on obstetric ultrasound as early as 19 weeks. It typically occurs in the adrenal gland (90%) and is usually stage 1, 2, or 4s (see Staging systems below). Metastases to the bone are rare. Hepatic metastases have been seen along with placental metastases. Fetal hydrops has been described secondary to placental metastases. Fetal neuroblastoma can cause preeclampsia in the mother secondary to catecholamine secretion.

The likelihood of surviving neuroblastoma is dependent of the age at diagnosis, site of the primary lesion, histologic tumor markers, and the stage of the malignancy. Patients younger than 1 year who have an extra-abdominal tumor at a low stage have a good prognosis.

Staging systems

Two staging systems are commonly in use today: the Evans and the International Neuroblastoma Staging System (INSS).

The Evans system consists of 5 stages:

• Stage 1: Tumor is confined to the organ of origin.
• Stage 2: Tumor extends beyond the organ of origin but does not cross midline. Ipsilateral regional lymph nodes may be involved.
• Stage 3: Tumor extends beyond the midline.
• Stage 4: Distant metastases are present.
• Stage 4s: Known as stage 4s, this last form occurs in infants who have a localized tumor that does not cross midline, with metastatic disease confined to the liver, skin, and bone marrow. No evidence of cortical bone involvement is observed.

The INSS takes into account surgical resectability, radiologic findings, and lymph node and bone marrow involvement.

• Stage 1: Localized tumor is confined to the organ of origin; complete resection with or without microscopic residual tumor; ipsilateral and contralateral lymph nodes are microscopically negative.
• Stage 2a: This involves a localized tumor with incomplete gross resection; ipsilateral and contralateral lymph nodes are microscopically negative.
• Stage 2b: This involves a unilateral tumor with incomplete or complete gross resection; ipsilateral lymph nodes are positive; contralateral lymph nodes are microscopically negative.
• Stage 3: In this stage, tumor crosses the midline with or without regional lymph node involvement, unilateral tumor is associated with positive contralateral lymph nodes, or a midline tumor is found with positive bilateral lymph nodes.
• Stage 4: Distant metastases are present.
• Stage 4s: This occurs in infants with a localized tumor that does not cross midline, with metastatic disease confined to the liver, skin, and bone marrow ( <10% tumor cells in bone marrow).

In general, patients with stage 1 disease have the best prognosis, whereas those with stage 4 disease have the worst. Interestingly enough, those with stage 4s neuroblastomas have a significantly better prognosis than those with stage 4 lesions despite its being considered a subclass of stage 4.

DIFFERENTIALS

Section 3 of 11  

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• Introduction
• Differentials
• Radiograph
• CT Scan
• MRI
• Ultrasound
• Nuclear Medicine
• Intervention
• Multimedia
• References

Other Problems to be Considered

Adrenal hemorrhage and Wilms tumor present as an abdominal mass.

Juvenile rheumatoid arthritis, leukemia, osteomyelitis, malabsorption and child abuse are included in the differential because of the variety of symptoms metastatic neuroblastoma can cause.

Adrenal carcinomas and pheochromocytoma are in the differential but are rare in this age group.

Primitive neuroectodermal tumor (PNET) may be considered, particularly paraspinal masses in children aged 5-10 years.

Esophageal duplication might be present in infants.

In neonates, infradiaphragmatic extralobar sequestration should be considered in the differential diagnosis.

RADIOGRAPH

Section 4 of 11  

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• Radiograph
• CT Scan
• MRI
• Ultrasound
• Nuclear Medicine
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• Multimedia
• References

Findings

Plain radiographs of the abdomen may show a flank mass. Stippled calcifications are present on up to 30% of radiographs. Hepatomegaly may occur secondary to metastatic involvement. Plain images of the chest often show a posterior mediastinal mass. Splaying of the ribs and rib erosion has been seen in patients with thoracic neuroblastomas due to the primary tumor. Pleural effusions and pleural nodules have been seen on chest radiographs. Lung parenchymal metastases are rarely seen on radiographs but often detected on autopsy. Widening of the paraspinal line can be seen secondary to retrocrural extension of retroperitoneal neuroblastomas.

Bone metastases usually occur in the long bones and typically present as irregular lucencies or lytic lesions in the metaphysis or submetaphyseal bone. Lytic lesions may be seen in skull, ribs, and pelvis. Sclerotic lesions have been seen and may be secondary to tumor infarction. Periosteal reaction is common. Widening of the cranial sutures secondary to dural metastasis can be seen in neuroblastoma. The classic hair-on-end appearance, albeit unusual in neuroblastoma, can be seen in the skull in destructive lesions.

Intraspinal extension of neuroblastomas can be seen on radiographs. Lateral views of the spine may show widening of the neuroforamina. Vertebral-body scalloping, erosion of the pedicles, and scoliosis have also been seen in patients with intraspinal involvement. Intraspinal involvement may be present in the absence of these findings and MRI is far superior in evaluating for intraspinal involvement.

Intravenous pyelography (IVP) and excretory urography were widely used in the past to evaluate patients with adrenal neuroblastomas before the advent of CT, MRI, and ultrasonography. Adrenal neuroblastomas typically displace the ipsilateral kidney laterally and downward, producing the classic drooping-lily sign on excretory urograms. The drooping lily sign is also caused by an obstructed upper moiety of a duplex collecting system.

CT SCAN

Section 5 of 11  

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• Introduction
• Differentials
• Radiograph
• CT Scan
• MRI
• Ultrasound
• Nuclear Medicine
• Intervention
• Multimedia
• References

Findings

CT is the modality most commonly used to diagnose and stage neuroblastomas. CT can show the organ of origin, extent of the tumor, lymphadenopathy, metastases, and calcifications. About 80-90% of neuroblastomas show stippled calcifications on CT.

Neuroblastomas often encase or compress adjacent blood vessels. Vessels that are commonly engulfed are the inferior vena cava, the renal veins and arteries, the splenic vein, the aorta, the celiac artery, and the superior mesenteric artery. Neuroblastomas rarely invade into the lumen of blood vessels.

The tumors often appear lobulated and typically have a heterogeneous appearance on contrast-enhanced CT. There are areas of low attenuation in the mass secondary to necrosis and hemorrhage. CT is good for detecting lung metastases and focal liver metastases (which appear as focal hypoattenuating and poorly enhancing masses). Bone-window settings should always also be examined to assess for skeletal metastases.

False Positives/Negatives

Diffuse liver metastases may be missed on CT. Cerebral metastases can appear as enhancing meninges secondary to dural metastases, which can simulate meningitis. Sometimes, brain metastases can appear as cystic lesions with peripheral enhancement, which can mimic an abscess. CT is poor for detecting metastatic disease to the bone and is limited in evaluating extradural extension of tumor into the spinal canal without the aid of intrathecal contrast material (CT myelography).

MRI

Section 6 of 11  

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• Introduction
• Differentials
• Radiograph
• CT Scan
• MRI
• Ultrasound
• Nuclear Medicine
• Intervention
• Multimedia
• References

Findings

MRI has a number of advantages over CT. One is that MRI does not use ionizing radiation. Other advantages include the multiplanar imaging capabilities of MRI and often, the elimination of the need to use intravenous contrast enhancement.

MRI utilizes the intrinsic tissue characteristics on T1- and T2-weighted imaging. Neuroblastomas are typically hypointense on T1-weighted images and hyperintense on T2-weighted images. When contrast material is administered, the tumor exhibits inhomogeneous enhancement. Calcifications appear as signal voids on MRIs. Hemorrhagic areas often appear bright on T1-weighted images.

Bone-marrow disease appears bright (hyperintense) and heterogeneous on T2-weighted images and dark (hypointense) on T1-weighted images. Diffuse liver metastases appear bright on T2-weighted MRIs.

Degree of Confidence

MRI is superior to CT in evaluating extradural extension of the tumor, and bone marrow involvement and in identifying diffuse hepatic metastases. MRI can show displacement of the spinal cord and/or nerve root displacement or compression and epidural spread of neuroblastoma exceptionally well. MRI results are well correlated with findings from bone-marrow biopsy.

ULTRASOUND

Section 7 of 11  

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• MRI
• Ultrasound
• Nuclear Medicine
• Intervention
• Multimedia
• References

Findings

Neuroblastomas appear as an inhomogeneously echogenic mass on sonograms. Calcifications typically appear as focal brightly echogenic areas in the mass. In masses with fine calcifications, images show diffuse, increased echogenicity. Acoustic shadowing from the calcifications may or may not be present. Hemorrhagic or necrotic areas in the tumor appear as hypoechoic or anechoic areas.

Ultrasonography can be used as a screening tool for detecting abdominal or pelvic masses in children. Doppler sonography can be used to identify blood flow through blood vessels encased or compressed by the tumor. Increased vascularity of neuroblastomas has been reported on Doppler sonograms, although typically most lesions show reduced vascularity.

Obstetric sonography can depict fetal neuroblastomas as early as 19 weeks' gestational age. Most of the cases diagnosed during obstetric ultrasonography are in the third trimester (around 36 weeks).

Ultrasonography is used to differentiate adrenal hemorrhage from neuroblastoma. Adrenal hemorrhage is the most common cause of adrenal mass in the neonatal population. They typically appear echogenic in the newborn, as neuroblastomas do, but they gradually become anechoic and avascular and often become smaller on serial sonograms as they regress.

Degree of Confidence

Sonograms can depict liver metastases, but they are limited in assessing the extent of metastatic disease to the liver. This is better evaluated with CT or MRI, though all these techniques may be complimentary, eg, a metastasis may be visible on sonography and not CT or vice versa.

False Positives/Negatives

As mentioned above, sonography can be used to identify liver metastases, but it is limited in assessing the extent of metastatic disease to the liver. This is better evaluated with CT or MRI.

NUCLEAR MEDICINE

Section 8 of 11  

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• MRI
• Ultrasound
• Nuclear Medicine
• Intervention
• Multimedia
• References

Findings

Bone scans obtained by using technetium-99m methylene diphosphate (MDP) are performed in many patients with neuroblastoma to assess metastatic disease. Approximately 74% of primary tumors in neuroblastoma take up ^99mTc MDP. Uptake may be seen in liver and lung metastases as well. ^99mTc MDP scans cannot be used to differentiate between cortical and bone marrow metastases; this limits their usefulness in accurately staging the disease, particularly in differentiating stage 4 from stage 4s.

Iodine-131 metaiodobenzylguanidine (MIBG) and iodine-123 MIBG are used to identify sites of primary neuroblastomas. Tumors that contain sympathetic tissue, such as neuroblastomas, ganglioneuroblastomas, ganglioneuromas, medullary thyroid carcinomas, pheochromocytomas, and carcinoids, take up MIBG. However, MIBG scanning cannot be used to differentiate these lesions. In the age group in which neuroblastoma (and its more benign forms, ganglioneuroblastoma and ganglioneuroma) is prevalent, the other tumors are rare.

MIBG has also been used to follow up the response to treatment in neuroblastoma patients. One of the drawbacks of using MIBG is that up to 30% of neuroblastomas may not take up MIBG, though 95% of neuroblastomas secrete catecholamines. Also, up to 50% of recurrent neuroblastomas do not take up MIBG even if they took up MIBG before therapy (Lonergan, 2002).

¹³¹I MIBG has a high principal proton energy (364 KeV). It emits beta particles, which gives a large dose of radiation to the patient. ¹²³I MIBG has a lower principal photon energy (159 KeV). It also does not emit beta particles, giving less radiation dose to the patient. ¹²³I MIBG has a shorter half-life (13 h) than that of ¹³¹I MIBG (8 d), and it must be used the day it is produced, making it more expensive and less readily available.

Another isotope that can be used in detecting primary neuroblastomas is indium-111 pentetreotide, which is a somatostatin analog. Studies have shown that it is as sensitive as MIBG in detecting neuroblastoma and other catecholamine-secreting tumors. ¹¹¹In pentetreotide has 2 principal photon energies (174 and 245 KeV), both of which are lower than that of ¹³¹I MIBG. It also does not emit beta particles, giving a lower radiation dose to the patient. ¹¹¹In pentetreotide also requires less patient preparation and produces images with better resolution than MIBG images on gamma scintillation cameras, but it is not widely used in pediatric oncology imaging.

Degree of Confidence

See Findings above.

False Positives/Negatives

See Findings above.

INTERVENTION

Section 9 of 11  

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• Introduction
• Differentials
• Radiograph
• CT Scan
• MRI
• Ultrasound
• Nuclear Medicine
• Intervention
• Multimedia
• References

The role for interventional radiology is controversial. Percutaneous biopsy can offer diagnostic and prognostic factors that are necessary to assign the patient to a low, intermediate or high-risk treatment protocol, such as DNA index, N-MYC status, and favorable or unfavorable histology. Dr Shimada, who defined the current histologic system, currently does not recognize the role of percutaneous needle biopsy for diagnosis (Shimada, 2001).

Radiofrequency ablation potentially can treat limited hepatic or bone metastases. However, hepatic metastases often regress with age or treatment and bone metastases can respond to chemotherapy or radiotherapy.

MULTIMEDIA

Section 10 of 11  

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• Differentials
• Radiograph
• CT Scan
• MRI
• Ultrasound
• Nuclear Medicine
• Intervention
• Multimedia
• References

Media file 1:  Axial nonenhanced T1-weighted MRI shows a hypointense mass in the retroperitoneum originating from the left adrenal gland. The mass displaces the left kidney in an anterolateral direction, it extends through the neuroforamen into the spinal canal, and it displaces the spinal cord to the right. The exact site of origin of large masses can be difficult to determine. Sympathetic-chain primaries supposedly invade the spinal canal with greater frequency than do adrenal primaries.
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Media file 2:  Axial T2-weighted MRI in the same patient as in Image 1 again demonstrates extradural extension into the spinal canal. The tumor appears hyperintense. Spinal cord displacement is better demonstrated on T2-weighted images than on other images.
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Media file 3:  Sagittal T2-weighted MRI in the same patient as in Images 1-2 shows a hyperintense extradural mass in the lower thoracic spine. Axial and coronal images confirm that this is extradural extension of a neuroblastoma of the left adrenal gland.
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Media file 4:  Coronal T2-weighted MRI in the same patient as in Images 1-3 shows a hyperintense mass in the left adrenal gland. The mass is extending cephalad into the spinal canal via the neuroforamen.
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Media type:  MRI

Media file 5:  Intravenous pyelogram (IVP) shows a classic drooping-lily sign involving the right kidney. This patient had a known right adrenal neuroblastoma.
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Media file 6:  Another intravenous pyelogram (IVP) shows an inferiorly displaced kidney on the right. Above the right kidney are stippled calcifications. These findings are consistent with those of a neuroblastoma.
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Media file 7:  This intravenous pyelogram (IVP) was obtained in a toddler who presented with abdominal pain and a palpable mass in the left flank. A drooping-lily sign is present on the left. The patient was referred for further workup.
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Media file 8:  Transverse sonogram of the left renal area was obtained in a patient whose intravenous pyelogram (IVP) is shown in Image 7. Sonogram shows an inhomogeneously hyperechoic, extrarenal mass that laterally displaces the kidney (which appears as a relatively hypoechoic structure).
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Media file 9:  Anteroposterior (AP) views of both knees show irregular lucencies in both distal femoral and proximal tibial metaphyses; these represent relatively symmetrical metastatic disease.
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Media file 10:  Image shows a destructive metastatic lesion involving the proximal fibular metaphysis with periosteal reaction in the proximal fibular diaphysis.
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Media file 11:  This patient has enhancing dural metastases near the frontal and occipital lobes. This finding could result in widening of the sagittal suture on plain images of the skull.
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Media file 12:  Classic hair-on-end appearance of a destructive metastatic lesion of the skull.
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Media file 13:  Frontal view of the chest shows a mass in the right thorax behind the heart. Posterior rib changes and the lateral view (Image 14) confirm that this is a posterior mediastinal mass. Note splaying and thinning of the ribs in the lower rib cage on the right. This was a thoracic neuroblastoma.
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Media file 14:  Lateral view of the chest in the patient in Images 13-17 confirms the posterior mediastinal mass.
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Media file 15:  Nonenhanced axial CT scan of the chest in a patient with a thoracic neuroblastoma (same patient as in Images 13-17) shows a large, right posterior mediastinal mass extending into the spinal canal and displacing the cord laterally to the left.
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Media file 16:  Axial T2-weighted chest MRI in the same patient as in Image 15, who had a thoracic neuroblastoma, shows a large, right posterior mediastinal mass extending into the spinal canal and displacing the cord laterally to the left. The mass is hyperintense on T2-weighted images.
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Media file 17:  Sagittal T2-weighted MRI of the chest in the same patient as in Image 16 shows a large, hyperintense, right posterior mediastinal mass extending into the spinal canal through multiple neuroforamina.
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Media file 18:  Anteroposterior (AP) or preorbital view of the skull shows widening of the sagittal and lambdoid sutures. This finding is due to dural metastases.
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Media file 19:  Lateral view of the skull shows widening of the coronal sutures and multiple lucencies in the parietal and frontal bones of the skull in this patient with metastatic neuroblastoma.
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Media file 20:  Axial CT scan of the orbits shows a heterogeneous-appearing, metastatic soft-tissue mass in the right orbit that displaces the globe and lateral rectus muscle medially. This patient presented with proptosis of the right eye.
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Media file 21:  Axial CT scan of the orbits in the patient in Image 20, obtained a few millimeters cephalic, show calcifications in the left orbital mass.
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Media file 22:  Bone window in a patient with bilateral proptosis shows osseous destruction involving both lateral orbital walls (left to right).
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Media file 23:  Bone-window axial CT study of the orbits (same patient as in Image 22) shows extensive bony destruction involving both frontal bones.
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Media file 24:  Coronal CT scan of the orbits and sinuses shows a large, enhancing, and expansile mass occupying the ethmoid air cells that is invading the cribriform plate and breaking through to the left anterior cranial fossa. This entity is known as an esthesioneuroblastoma. Image courtesy of Michael Lev, MD.
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Media file 25:  Technetium-99m methylene diphosphate (MDP) bone scan shows a focus of intense activity in the left lower quadrant of the abdomen adjacent to the spine, above the bladder. This finding corresponds to a neuroblastoma in this location. Image shows activity in the dilated renal calyces on the left, which suggests partial obstruction of the left ureter by the mass. No evidence of metastatic disease is observed.
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Media type:  Image

REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Differentials
• Radiograph
• CT Scan
• MRI
• Ultrasound
• Nuclear Medicine
• Intervention
• Multimedia
• References

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• Blickman H. Pediatric Radiology: The Requisites. 2nd ed. 1998: 177-9.
• Currarino G. The Genitourinary Tract. In: Silverman FN, Kuhn JP, eds. Essentials of Caffey's Pediatric X-Ray Diagnosis. 1990: 724-9.
• Donnelly LF. Fundamentals of Pediatric Radiology. 2001: 163-5.
• Hoffer FA, Chung T, Diller L. Percutaneous biopsy for prognostic testing of neuroblastoma. Radiology. 1996;200:213-6.
• Lonergan GJ, Schwab CM, Suarez ES. Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma: radiologic-pathologic correlation. Radiographics. Jul-Aug 2002;22(4):911-34. [Medline].
• McHugh K, Pritchard J. Problems in the imaging of three common paediatric solid tumours. Eur J Radiol. Feb 2001;37(2):72-8. [Medline].
• Schilling FH, Spix C, Berthold F. Neuroblastoma screening at one year of age. N Engl J Med. Apr 4 2002;346(14):1047-53. [Medline].
• Schofield B, Cotran RS. Diseases of Infancy and Childhood. In: Cotran RS, Kumar V, Robbins SL, eds. Robbins Pathologic Basis of Disease. 5th ed. 1994: 459-61.
• Shimada H, Umehara S,, Monobe Y. International neuroblastoma pathology classification for prognostic evaluation of patients with peripheral neuroblastic tumors: a report from the Children's Cancer Group. Cancer. 2001;92:2451-61. [Medline].
• Sofka CM, Semelka RC, Kelekis NL, et al. Magnetic resonance imaging of neuroblastoma using current techniques. Magn Reson Imaging. Feb 1999;17(2):193-8. [Medline].
• Thrall JH, Ziessman HA. Nuclear Medicine: The Requisites. 1995: 47-8.
• Woods WG, Gao RN, Shuster JJ. Screening of infants and mortality due to neuroblastoma. N Engl J Med. Apr 4 2002;346(14):1041-6. [Medline].

Article Last Updated: Sep 9, 2005