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Neuroblastoma (Detailed)

Most common extracranial solid tumor of childhood 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.

References (5)

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 (Detailed)?

Neuroblastoma arises from primitive neural crest cells that form the adrenal medulla and sympathetic ganglia. It is the most common cancer in infants and accounts for 8-10% of pediatric malignancies; median age at diagnosis is 17 months. Tumors most often occur in the adrenal gland (40%) but may originate anywhere along the sympathetic chain (paraspinal, mediastinal, cervical).

Biologic and clinical heterogeneity defines management. Risk stratification incorporates age, MYCN amplification, INSS/INRGSS stage, histology, ploidy, and segmental chromosomal aberrations (1p, 11q deletions). MYCN amplification (occurring in 20-25% of cases) confers high risk regardless of stage. Specific subtypes (Stage 4S in infants) can spontaneously regress.

Treatment is multimodal and risk-adapted: low-risk disease may need only observation or surgery; intermediate-risk receives moderate-dose chemotherapy plus surgery; high-risk requires intensive multimodal therapy — induction chemotherapy, surgical resection, myeloablative consolidation with autologous stem cell transplant, radiotherapy, and post-consolidation immunotherapy with anti-GD2 antibodies (dinutuximab) plus cis-retinoic acid maintenance. Long-term survival ranges from 95% in low-risk to 50-60% in high-risk disease.

Symptoms

Abdominal mass or distension (most common)
Pain, irritability, or fatigue
Bone pain from metastatic disease
Periorbital ecchymoses ("raccoon eyes")
Horner syndrome with cervical/upper thoracic tumors
Opsoclonus-myoclonus-ataxia (paraneoplastic)
Hypertension from catecholamine secretion
Watery diarrhea from VIP-secreting tumors

Risk Factors

Familial neuroblastoma (germline ALK or PHOX2B mutations)
Hirschsprung disease, central hypoventilation syndrome (PHOX2B)
Neurofibromatosis type 1, Beckwith-Wiedemann syndrome
MYCN amplification (somatic, high-risk marker)
Diploid DNA content
Segmental chromosomal aberrations (1p, 11q loss; 17q gain)
Age >18 months at diagnosis
Unfavorable histology (poorly differentiated, high MKI)

When to See a Doctor?

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

  • Palpable abdominal mass in a young child
  • Bone pain or limp without trauma
  • Periorbital ecchymoses or proptosis
  • Horner syndrome in a child
  • Opsoclonus-myoclonus-ataxia
  • Catecholamine excess symptoms (hypertension, sweating)

Treatment Methods

01
Risk-adapted multimodal therapy based on COG/SIOPEN guidelines
02
Low-risk: observation or surgery alone
03
Intermediate-risk: moderate-dose chemotherapy and surgery
04
High-risk: induction chemotherapy, surgery, myeloablative therapy with autologous stem cell rescue, radiotherapy
05
Anti-GD2 immunotherapy (dinutuximab) with GM-CSF, IL-2, isotretinoin maintenance
06
Targeted therapy for ALK-mutant disease (crizotinib, lorlatinib in trials)
07
MIBG radiotherapy for relapsed/refractory disease
08
Long-term survivorship care for late effects

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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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.