Ch033_X3251.

qxd 20/9/06 3:37 PM Page 255

CHAPTER 33 NEUROBLASTOMA 255

33 Neuroblastoma
KEY CLINICAL AND IMAGING POINTS

Neuroblastoma arises from the embryonal neural crest. There

is a spectrum of disease ranging from well-differentiated
ganglioneuroma to highly malignant undifferentiated
neuroblastoma. These tumors arise along the sympathetic
neural axis, most frequently in the adrenal glands, although
cervical, thoracic, and pelvic neuroblastoma may also be
encountered. The peak incidence is about 2 years of age.
Children with an abdominal primary tumor who are older
than 1 year of age have metastatic disease at presentation in
approximately 75% of cases. Patients with localized disease,
who form the minority of patients, may be treated with
surgery alone; other patients receive neoadjuvant
chemotherapy after initial staging and require repeat imaging
prior to surgery. Neuroblastoma in the first year of life,
which may be diagnosed on antenatal sonography, behaves
in a less aggressive manner than in older children and carries
a relatively good prognosis.

Initial Staging
• The first investigation of an abdominal mass is with
ultrasound. However, tumor at other sites (such as the
thorax) may be better imaged with CT or MRI.
• CT with contrast or MRI can be used to demonstrate local
tumor extent and vascular encasement.
• MRI is the most sensitive technique for detecting focal and
diffuse abnormalities in bone marrow. CT can also
demonstrate bony involvement when changes are gross.
• Bone scintigraphy with 99mTc MDP is used to detect
osseous lesions.
• Meta-iodobenzylguanidine (MIBG) localizes in amine-
secreting tumors; radionuclide scanning with MIBG is
performed routinely in all patients at diagnosis.
• I131 or I123 MIBG scintigraphy is helpful for detection of
skeletal and nonskeletal lesions.
• 18F-FDG PET is useful in staging and monitoring response
to treatment of MIBG-negative tumors.
Ch033_X3251.qxd 20/9/06 3:37 PM Page 256

256 PEDIATRIC TUMORS

䊊 Pitfall: Findings at MIBG can be false positive in the

kidney, bowel, urinary bladder, brown fat (shoulders),
and when showing diffuse lung activity.

CT and MRI Tips

• Loss of tissue planes may help to distinguish
neuroblastoma from Wilms’ tumor.
• Tumor is infiltrating and difficult to measure.
• Calcification is better shown on CT.
• T2W sequences are required to assess spinal involvement.
• MRI is better than CT at demonstrating extension of
abdominal and thoracic tumor into neural foramina and
spinal canal.
• Contrast-enhanced CT can identify intraspinal tumor
extension.

Preoperative Staging after Neoadjuvant Therapy

Neuroblastoma is expected to respond to neoadjuvant
chemotherapy; the most malignant tumors (as assessed
histologically) tend to demonstrate the greatest reduction
in tumor bulk. Preoperative assessment of abdominal
and thoracic tumors should be undertaken with CT or
MRI.

KEY REPORT ELEMENTS

T: Primary Tumor
• Size of tumor (perpendicular axial diameters and length)
• Confirm organ of origin
• Local extent:
䊊 Presence of vascular encasement
䊊 Extension of tumor across the midline
䊊 Assess for presence of extension into neural foramina or
spinal canal (“dumbbell” tumor)
䊊 Identify bony erosion by primary tumor
䊊 Identify invasion of adjacent organs (e.g., kidney, liver,
spleen, diaphragm)

N: Lymph Nodes
• Presence of nodal enlargement in retroperitoneum,
retrocrural region, or mediastinum
• Regional nodal drainage depends on the specific site of
tumor origin.
Ch033_X3251.qxd 20/9/06 3:37 PM Page 257

CHAPTER 33 NEUROBLASTOMA 257

A large tumor (T) is present in the right retroperitoneum, with

adenopathy (N) displacing the aorta (black arrow), inferior vena
cava (arrowheads), and splenic vein (white arrow). Fine
calcifications are evident within the tumor.

M: Metastases
• Assess for bone metastases.
• Pulmonary metastases are rare.
• Hepatic metastatic disease is commonly seen in stage IVS
disease in patients younger than 1 year old.

STAGING SYSTEM

The International Neuroblastoma Staging System (INSS)

is the most widely used staging classification. It takes into
account radiologic findings, surgical resectability, and
pathologic status of lymph nodes and bone marrow.

Stage I Localized tumor with complete macroscopic

excision (with or without microscopic residual
disease)
Stage IIA Localized tumor with incomplete gross
excision and ipsilateral lymph nodes negative
for tumor microscopically
Ch033_X3251.qxd 20/9/06 3:37 PM Page 258

258 PEDIATRIC TUMORS

Stage IIB Localized tumor with or without complete

gross excision with ipsilateral lymph nodes
positive for tumor
Stage III Tumor infiltrating across the midline with or
without regional node involvement, or
localized unilateral tumor with contralateral
lymph nodes positive for tumor, or midline
tumor with bilateral extension by infiltration
or lymph node involvement
Stage IV Any primary tumor with dissemination to
distant lymph nodes, bone, bone marrow,
liver, or other sites
Stage IVS Localized primary tumor (i.e., stages I, IIA, or IIB)
with dissemination limited to skin, liver, or bone
marrow in patients under the age of 1 year

TREATMENT IMPLICATIONS OF IMAGING FINDINGS

• Tumors deemed unresectable at initial staging (e.g., tumors

encasing major vessels, invasion of the spinal canal, large
tumors crossing the midline) are treated with
chemotherapy.
• CT is useful for assessing tumor response and resectability
post-therapy.
• Persistent MIBG activity after induction therapy portends
a poor outcome.

SELECTED REFERENCES
Boubaker A, et al: Nuclear medicine procedures and neuroblastoma
in childhood: their value in the diagnosis, staging and
assessment of response to therapy. Q J Nucl Med 47:31–40, 2003.
Lonergan GJ, et al: Neuroblastoma, ganglioneuroblastoma, and
ganglioneuroma: radiologic-pathologic correlation.
RadioGraphics 22:911–934, 2002.
Meyer JS, et al: Imaging of neuroblastoma and Wilms’ tumor. Magn
Res Imaging Clin North Am 10:275–302, 2002.
Siegel MJ: Neuroblastoma. In Husband JE, Reznek RH (eds): Imaging
in Oncology, 2nd ed. London, Taylor & Francis, 2004, pp 953–971.
Siegel MJ, et al: Staging of neuroblastoma at imaging: report of
the radiology diagnostic oncology group. Radiology
223:168–175, 2002.