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Neuroblastoma

An aggressive pediatric solid tumor arising from sympathetic nervous system precursors.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

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This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Onkoloji department. Book Appointment →

What is Neuroblastoma?

Neuroblastoma is an embryonal malignancy of the sympathetic nervous system arising from neural crest cells. It is the most common extracranial solid tumor in childhood, accounting for approximately 8-10% of all pediatric cancers and about 15% of cancer-related deaths in children. The median age at diagnosis is 17-18 months and 90% of cases occur before age 5.

The tumor most commonly arises in the adrenal medulla (40%) but can develop anywhere along the sympathetic chain, including the abdomen, chest, neck, and pelvis. Neuroblastomas exhibit remarkable biological heterogeneity, ranging from spontaneous regression in infants to highly aggressive metastatic disease in older children.

Risk stratification incorporates age, stage (INRG/INSS), histology, MYCN amplification status, DNA ploidy, and 11q aberrations. MYCN amplification, found in 20-25% of cases, is the strongest adverse prognostic marker. High-risk neuroblastoma requires multimodal therapy including induction chemotherapy, surgery, autologous stem cell transplant, radiation, immunotherapy with anti-GD2 antibody, and differentiation therapy with isotretinoin.

Symptoms

Abdominal mass or distension (most common presentation)
Bone pain or limping (from metastases)
Periorbital ecchymoses (raccoon eyes from skull base metastases)
Proptosis and orbital swelling
Subcutaneous nodules (blueberry muffin appearance in infants)
Hepatomegaly with respiratory distress (stage MS in infants)
Horner syndrome (cervical/thoracic tumors)
Opsoclonus-myoclonus-ataxia syndrome (paraneoplastic)
Hypertension or watery diarrhea (catecholamine/VIP secretion)
Spinal cord compression from dumbbell tumors

Risk Factors

Young age (median 17-18 months at diagnosis)
Familial neuroblastoma (ALK or PHOX2B germline mutations, rare)
Hirschsprung disease, congenital central hypoventilation (PHOX2B-related)
Beckwith-Wiedemann syndrome
Neurofibromatosis type 1
DICER1 syndrome
Prenatal exposure to certain medications (limited evidence)
Most cases are sporadic with no identifiable predisposing factor

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Persistent abdominal mass or distension in a young child
  • Unexplained bone pain or limping
  • Periorbital bruising without trauma
  • Persistent fever, weight loss, or fatigue
  • Skin nodules in an infant
  • Sudden onset opsoclonus-myoclonus (dancing eyes, dancing feet)
  • New onset Horner syndrome in a child
  • Suspected spinal cord compression (back pain, weakness)
  • Hepatomegaly with respiratory distress in infants

Treatment Methods

01
Risk stratification: low, intermediate, or high risk based on INRG criteria
02
Observation: select stage MS infants with favorable biology may regress spontaneously
03
Surgery alone: for low-risk localized tumors
04
Moderate chemotherapy: carboplatin, etoposide, doxorubicin, cyclophosphamide for intermediate risk
05
High-risk multimodal therapy: induction chemotherapy with intensified regimens
06
Tumor resection after induction chemotherapy
07
Myeloablative consolidation with autologous stem cell rescue (often tandem)
08
External beam radiation to primary site and persistent metastatic sites
09
Anti-GD2 immunotherapy (dinutuximab) with GM-CSF, IL-2
10
Differentiation therapy with 13-cis-retinoic acid (isotretinoin) maintenance
11
Targeted therapy: ALK inhibitors (crizotinib, lorlatinib) for ALK-mutated tumors
12
MIBG radiotherapy for relapsed/refractory disease
13
Long-term follow-up for late effects: hearing loss, growth, cardiac, secondary cancers

Which Department to Visit?

You can visit our Onkoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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