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Neuroblastoma Staging Symptoms Causes Diagnostic Tests and Treatment

Neuroblastoma Staging Symptoms Causes Diagnostic Tests and Treatment

Posted By HealthcareOnTime Team Posted on 2021-08-23

Neuroblastoma - a rare cancer of the developing sympathetic nervous system, is derived from the neuroblasts. The incidences of childhood cancer in India are lower than that in developed countries. Yet, considering that a new life is battling for its survival, 30 to 124 cases per 10 lakh children is not an insignificant number. Neuroblastoma is a solid tumour in children that often originates from the adrenal gland and spreads around the spine, chest, abdomen, bone marrow and liver. In very rare cases, the cancer spreads to the skin during infancy. This neoplasm is mostly asymptomatic, but widespread at the time of birth

Neuroblastoma Staging Symptoms Causes Diagnostic Tests and Treatment

Neuroblastoma Staging System - To Classify The Disease
Neuroblastoma is classified into different stages that aid in determining its prognosis. Staging specifies whether the cancer has spread and if yes, how far. This also helps to decide the possible treatment options.

Stage 1 Tumour at this stage is completely resectable i.e removable by surgery. The cancer has not spread to other parts of the body and remains restricted to the primary site of occurrence only.

Stage 2 Tumour at this stage cannot be completely removed by surgery. It has not spread across the mid-line of the body (spinel, but depending on the involvement of lymph nodes, it is divided into two types: stage 2A and 2B.

Stage 2 A The lymph nodes present on the tumour side do not contain the cancerous tumour cells.

Stage 2 B The lymph nodes present on the tumour side do contain the cancerous tumour cells, while the lymph nodes on the other side of the tumour do not comprise of tumour cells.

Stage 3 This stage includes tumours that have spread across the spine to the contralateral side or have contralateral nodes involved. At this stage, the tumour cannot be completely resected by surgery.

Stage 4S This is the stage where the cancer cells have spread from the initial site to other parts, i.e from spine to the liver, skin and minimally to the bone marrow. This stage occurs mostly in newborns below 1 year of age. Stage 4S is also termed as "special neuroblastoma".

Stage 4 Here the cancer cells have metastasized to the distant lymph nodes and organs which do not fulfill 45 stage.

Neural and Non-neural Mutual Associations
A variety of birth and developmental defects are present in association with neuroblastoma. These can be either neural or non-neural conditions. Neuroblastoma tumour can be associated with maldevelopment of neural crest tissues. This is evident by:
- Occurrence of Neuroblastoma tumours along with Von Recklinghausen disease, which manifests as development of noncancerous tumours on nerve and skin
- Severe hypoglycemia, etc.
Neuroblastoma also occurs in association with non-neural crest syndromes such as:
- Beckwith-Wiedemann syndrome (overgrowth disorder)
CHARGE syndrome - a condition which shows multiple features like heart defects, growth retardation, genital abnormalities, ear abnormalities, etc

Fetal and Infant Traits Neuroblastoma is a malignant tumour representing a mass on abdomen, neck, and other body parts. The clinical manifestations of neuroblastoma depend upon the affected location and include the following:
In the abdomen
- Swollen Abdomen (Abdominal mass and pain)
- Scrotal Mass

In the chest
- Inability to move, paralysis, weakness (cancers compressing spinal cord)
- Breathing difficulties (due to tumour on chest)
- Hydrops Fetalis (abnormal accumulation of fluid in fetal compartments)
- Unequal pupils, drooping eyelids, nerve damage in neck (Horner's syndrome)
Other signs-
- Cutaneous metastasis (cancer growth on skin)
- Neurogenic Bladder (bladder dysfunction)
- Congenital chylothorax (accumulation of lymphatic fluid in pleural cavity)
- Myoclonic encephalopathy (seizures, jerks, muscle twitches)
- Bronchial obstruction from mediastinal tumour
' Bone pain

These neoplastic tumours are usually found as an unsuspected detection in sonographs in prenatal testings.

It is important to visit the pediatrician if you notice below symptoms
- Bulging eyes
- Dark circles around the eyes
- Bluish lumps under the skin
- Lumps in abdomen, neck, chest
- Easy bruising/bleeding
- Petechiae (red or purple spots on skin caused due to broken capillary blood vessel of bleeding)
- Droopy eyelids, smaller pupils (Horner syndrome)
- Uncontrolled eye movements

Genetic Defects
Neuroblastoma tumour cells exhibit the following genetic features:
- Deletions of short arm of Chromosome 1
- Deletions of Long arm of Chromosome 119
- Extrachromosomal double minute chromatin bodies
- Homogeneously staining regions amplified with N- mycgene
Neuroblastoma and N-myc amplification co-relations:
- Neuroblastoma cells which are found to have amplification of N-myc gene also show abnormal translocation of 17q chromosome.
- Patients in later stages of cancer show higher amplifications of both the oncogenes N-myc and ras.
- The loss of heterozygosity of chromosome 1p is associated with N-myc amplification.
- N-myc amplification is accountable for abnormal histological patterns of the tumour cells.

N-myc amplification can evaluate increased expression of Multidrug Resistant-Associated Protein (MDRAP) gene, accounting for reduced survival. The estimation of N-myc can therefore aid in the measurement of magnitude of the disease depending upon the stage of the tumour, as Stages 1 and 4S conveys single copy of N-myc gene, and Stages 3 and 4 display multiple copies due to genomic amplification.

How was your child diagnosed with neuroblastoma?
A. Blood and Urine Tests
1. Catecholamine Analysis - Most of the neuroblastomas which are chemically active, secrete catecholamines (vanillmandelic - acid and homovanillic acid mainly) in excess amounts than produced by normal sympathetic nerve cells. Urinary catecholamine analysis will aid in diagnosing neoplastic tumour and can also help detec the possibility of recurrence.

2. Serum Ferritin - Cells of Neuroblastoma secrete ferritin, elevating the serum ferritin levels which interfere with the immune function of the lymphocytes.

3. Serum Lactate Dehydrogenase (LDH) - LDH aids in measuring tumour activity. Those with elevated levels of LDH may have metastasis and tumour recurrences.

4. DNA Cytometric Analysis (DNA index) - Tumour cells with aneuploidy have been associated with lower stage of disease and are likely to support the initial stages of the treatment. It helps to measure the percentage of tumour cells in the different phases of the cell cycle, also allowing prediction of its prognosis.

B. Imaging Tests These techniques help produce images and diagnose masses in chest mediastinal masses), abdomen (flank masses), ribs (rib erosion) due to primary neuroblastoma tumours, bone metastasis, lytic lesions in skull, ribs, pelvis, etc. They also help to measure the intraspinal extensions of neuroblastoma. The imaging techniques that aid in diagnosis include:

1. Computed Tomography (PET-CT Scan) - It is a device that uses X-rays to capture cross-sectional images of the different parts of the body. The CT scanner takes rotational images from different angles (horizontal and axial) instead of a single image. It helps in staging the tumours of neuroblastoma and also helps identify its organ of origin.

2. Magnetic Resonance Imaging (MRI) - Has the property of multiplanar imaging. It helps to produce images of soft tissues, especially of the brain and spinal cord. The advantage of MRI scan is that it does not use ionizing radiation. However, it cannot be used for children with kidney diseases.

3."1 or "1-Metaiodobenzylguanidine ("l-mIBG) Imaging-mIBG is a drug which is selectively taken up by neural crest tumours such as neuroblastoma. ""'lmIBG can also be used to give radionuclide therapy in patients with widespread disease.

4. Tc-MDP (Methylenediphonate) Bone Scans - It helps to identify the different bones which are affected by tumour.

5. Ultrasonography - Neuroblastoma tumours appear as echogenic masses which lack homogeneity. It helps in detecting tumours in abdomen and pelvic region of the children. It also helps to assess the size and extent of spread within the abdomen.

6. Positron Emission Tomography (PET) Scan. Fluorodeoxyglucose (FDG) is a glucose analog which is taken up by the cancer cells. FDG PET-CT Scan not only help assess the spread of tumour to bones and abdomen but also to other organs such as lungs, skin, bone marrow and brain.

C. Biopsies It is a procedure by which small samples of tissues are collected and studied under microscope. The samples are also sent for genetic testing.

Management and Treatment for Neuroblastoma
Neuroblastoma is curable but the survival rate depends on the child's age, location of tumour and stage of the tumour. Depending upon this, the survival is approximated into different risk groups as below:

- Low risk groups (Stage 1 and 2) - Survival rate is higher than 95%.
- Intermediate risk group (Stage 3) - Survival rate is about 90-95%.
- High risk group (stage 4) - Survival rate is lowered to around 40-50%
The different types of treatments available include, surgery, radiation therapy, chemotherapy, targeted therapy, iodine 131-miBG therapy, immunotherapy, etc.
- Surgery is done in neuroblastoma to remove primary tumour mass and regional lymph nodes.
- Radiation therapy is used to kill cancer cells so as to prevent them from spreading. It is mostly used for children who belong to lower risk groups.
- "'l-mlBG therapy is usually given to patients with widespread disease. mlBG is selectively taken up by cancer cells. The radioactive iodine (L-131) which is tagged to it emits beta radiations which kills the cancer cells.
- Chemotherapy involves administration of drugs in vein which circulate in the body and kill the cancer cells.

Finding the ray of hope?
Cancer is like a nightmare that puts you into the clutches of grief and affliction, and what can pull you back into the battle called life is a 'ray of hope'. Nobody can understand the depth of sorrow and the amount of confidence and strength required to fight back, nobody but the one who has been through it. What keeps you going in the times of distress is the never giving up attitude, the family that stands along the ups and downs, the conviction one gains from the knowledge and awareness and advanced science technology that will guide you in every step through this journey. Ray of hope lies just one step ahead.


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