Neuroblastoma

WHAT IS NEUROBLASTOMA?                                                                                                   

• NEUROBLASTOMA arises from the sympathetic nervous system: in the adrenal gland and sympathetic nervous system anywhere from neck to pelvic cavity. It is commonly diagnosed at around 20 months of age and mostly occurs below 5 years of age. This tumor has a variable clinical course from benign to malignant counterpart where outcomes are dismal.

WHY DOES NEUROBLASTOMA OCCUR?                                                                                                   

• Most of the Neuroblastomas are sporadic, about 1-2 % percent of tumors are familial and they can have multifocal disease and present early in life.

WHAT ARE THE SYMPTOMS OR RED FLAGS AND WHEN SHOULD YOU MEET THE DOCTOR?

• The symptoms or red flags depend on what site the tumor arises-
  • Neck - palpable swelling on the side of the neck.
  • Thorax – frequent respiratory infections.
  • Abdomen- pain, distension, hard mass palpable.
  • Pelvis- Urinary dysfunction, Constipation or fecal incontinence.
  • Para spinal tumor- Back pain (radicular), Scoliosis
  • Spinal cord compression/paraplegia.

• Some additional symptoms can be:-
  • Hypertension. ( increased blood pressure)
  • Weight loss, Anorexia, fever.
  • Bone pain due to bone metastasis.
  • Periorbital Ecchymosis (raccoon eyes/panda eyes).
  • Opsomyoclonus syndrome (OMAS)-dancing eyes and jerky (involuntary) movements.
  • VIP syndrome (Kerner-Morrison syndrome)-intractable diarrhoea.
  • The parents of children need to be very vigilant and observant. Doctor consultation is a must even if a few of the above symptoms are present in the child.

HOW DO WE DIAGNOSE NEUROBLASTOMA?                                                                                              

• Neuroblastoma can be diagnosed by conducting the following tests:-

  • Laboratory tests

  • Routine blood investigations.

  • Urinary VMA levels -24hours/spot.
  • CECT/MRI depending on local site.
  • MIBG/PET-CT for metastatic workup.
  • Bilateral bone marrow aspiration and biopsy.
  • Tissue diagnosis- Image guided biopsy from the tumor or if it is a non - metastatic small tumor, we can do upfront surgery. It would include Histopathology, IHC, N-Myc amplification and chromosomal aberrations.

WHAT DOES RISK STRATIFICATION MEAN?                                                                                                   

• Like most of the paediatric tumors, Neuroblastoma are staged and risk stratified before starting the treatment. This involves age, local stage, and metastatic disease, type of tumor, chromosomal aberration and N-Myc amplification. The risk stratification helps to define treatment and outcome.

WHAT ALL TREATMENT WILL MY CHILD UNDERGO FOR NEUROBLASTOMA?

• Low risk disease might only need observation or only surgery depending on the location and presence or absence of life threatening symptoms. The intermediate and high risk the treatment in intense and high risk includes bone marrow transplant as well.

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