Neuroblastoma
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Staging
The “International Neuroblastoma Staging System” (INSS) according to its anatomical presence at diagnosis:
- Stage 1: Localized tumor confined to the area of origin.
- Stage 2A: Unilateral tumor with incomplete gross resection; identifiable ipsilateral and contralateral lymph node negative for tumor.
- Stage 2B: Unilateral tumor with complete or incomplete gross resection; with ipsilateral lymph node positive for tumor; identifiable contralateral lymph node negative for tumor.
- Stage 3: Tumor infiltrating across midline with or without regional lymph node involvement; or unilateral tumor with contralateral lymph node involvement; or midline tumor with bilateral lymph node involvement.
- Stage 4: Dissemination of tumor to distant lymph nodes, bone marrow, bone, liver, or other organs except as defined by Stage 4S.
- Stage 4S: Age <1 year old with localized primary tumor as defined in Stage 1 or 2, with dissemination limited to liver, skin, or bone marrow (less than 10 percent of nucleated bone marrow cells are tumors).
The new INRG risk assignment will classify neuroblastoma at diagnosis based on a new International Neuroblastoma Risk Group Staging System (INRGSS):
- Stage L1: Localized disease without image-defined risk factors.
- Stage L2: Localized disease with image-defined risk factors.
- Stage M: Metastatic disease.
- Stage MS: Metastatic disease “special” where MS is equivalent to stage 4S.
Investigations :
Screening : Urine catecholamine level, Urine vanillylmandelic acid (VMA)
Imaging: Meta-iodobenzylguanidine scan, F-FDG-PET Scan
Tissue Biopsy : Microscopy : small, round and blue, and rosette patterns (Homer Wright pseudorosettes)
Prognosis :
The overall 5-year survival rate of neuroblastoma is 67%.
Treatment :
- Low-risk disease can frequently be observed without any treatment at all or cured with surgery alone.
- Intermediate-risk disease is treated with surgery and chemotherapy.
- High-risk neuroblastoma is treated with intensive chemotherapy, surgery, radiation therapy, bone marrow / hematopoietic stem cell transplantation,biological-based therapy with 13-cis-retinoic acid (isotretinoin or Accutane) and antibody therapy usually administered with the cytokines GM-CSF and IL-2.