Neuroblastoma natural history

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Haytham Allaham, M.D. [2]

Overview

If left untreated, patients with neuroblastoma may progress to develop fatigue, loss of appetite, joint pain, and fever. Complications of neuroblastoma may include persistent refractory diarrhea, Horner's syndrome, and transverse myelopathy. Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. However, the prognosis of neuroblastoma is generally regarded as poor.

Natural History

• Neuroblastoma patients may initially present with vague symptoms making the diagnosis of such patients difficult.
• If left untreated, patients with neuroblastoma may progress to develop fatigue, loss of appetite, joint pain, and fever.
• Gradual development of site specific symptoms may occur as the tumor size gradually increases.

Complications

• Complications of neuroblastoma may include:

• Persistent refractory diarrhea (due to vasoactive intestinal peptide secretion)
• Horner's syndrome (due to cervical sympathetic chain compression)
• Opsoclonus myoclonus syndrome and ataxia (due to a paraneoplastic syndrome)
• Hypertension (due to either catecholamine secretion or renal artery compression)
• Transverse myelopathy (due to tumor spinal cord compression)
• Anemia and suppressed immunity (due to bone marrow metastasis)

Prognosis

• Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. However, the prognosis of neuroblastoma is generally regarded as poor.
• Neuroblastoma INSS stage 1, stage 2, or stage 4S are associated with a 3 year survival rate of 75-90%.
• Neuroblastoma INSS stage 3 is associated with a 3 year survival rate of 80-90% among patients younger than 1 year of age.
• Neuroblastoma INSS stage 3 is associated with a 3 year survival rate of 50% among patients older than 1 year of age.
• Neuroblastoma INSS stage 4 is associated with a 3 year survival rate of 60-75% among patients younger than 1 year of age.
• Neuroblastoma INSS stage 4 is associated with a 3 year survival rate of 15% among patients older than 1 year of age.
• The table below lists the prognostic factors for neuroblastoma patients:

Prognostic Factor	Description
Patients Age	Patients older than 18 months of age are associated with poor prognosis.
Tumor Stage	Advanced INSS stages of neuroblastoma are associated with poor prognosis.
Tumor Grade	An unfavorable Shimada histology index is associated with poor prognosis.
Schwannnian Stroma	A reduced schwannian stroma background content on histological analysis is associated with poor prognosis.
Mitotic-karyorrhectic Index	A high mitotic-karyorrhectic index is associated with poor prognosis.
Genetics Mutations	Chromosome 1p deletion, chromosome 11q deletion, and N-MYC oncogene amplification are associated with poor prognosis.
Response to Treatment	Patients whose neuroblastoma responds to treatment and goes into complete remission have a better prognosis than people whose cancer does not respond to the initial treatment.