Neuroblastoma

Aaryanna Brown
What is a Neuroblastoma?

Neuroblastomas are tumors of the sympathetic ganglia and the adrenal medulla that are derived from the neural
crest cells and secrete catecholamines. These are usually seen in children and infants under the age of 5 and
usually diagnosed at 21 months. This is also the most common tumor seen in the first year of life. Many of these
start growing in utero
This is usually caused by chromosomal abnormalities such as the oncogene call MYCN. Due to its
amplification, this is found as a cause in about 25% of these cases and correlates with a high risk and poor
prognosis.
History and Physical Findings

Presenting Complaint and HPC:

Symptoms typically present depending on the location of the tumor.
1.Loss of appetite
2.Watering diarrhea
3.Vomiting
4.Weight loss
5.Fatigue
6.Bruising of the eyes
7.Bone pain
8.Fever
9.Weakness
10.
Limping
11.
Paralysis of the bladder and bowel dysfunction
History and Physical Examination

PMH: previous congenital birth defects may previously dispose this

Drug History: Patient is usually given NSAIDS’s but they do not relieve the pain.
Family History: There are two forms of this disease such as familial and sporadic. Children with familial forms
may be seen in different organs. However, there is a 1-2% of inheritance of this disease.
Physical Examination

Inspection:
 Patient looks fatigued
 Pallor
 Ecchymosis around the eyes
 Ptosis
 Nystagmus
 Muscle jerks
 Subcutaneous nodules
Physical Examination

Palpation
 Hepatomegaly
 Large, irregular mass that may seem to be crossing the midline
 Abdominal distention

Auscultation:
 Listen to the heart for signs of tachycardia and an irregular rhythm
 Blood pressure should also be taken
Physical Examination
Relevant Investigations

 Labs: these measure catecholamine metabolites such as VMA which is a metabolite form of NE and Epinephrine. HVA is also useful and is a
metabolite of Dopamine. Measuring free catecholamines may not be as useful as they are metabolized within the tumor before entering
systemic circulation. This is detected in the urine
 CBC: should be obtained to determine if the child has a pancytopenia
 PT/PTT: abnormalities in coagulation due to liver involvement
 ESR: it may be elevated in this case
 LDH and ferritin: these may indicate levels
 Imaging Studies: abdominal radiography of the kidneys, ureters, and bladder. CT and MRI can be done, however, an ultrasound would be
preferred first as sedation is required in most children. Therefore, a CT and an MRI could be invasive.
 Histology
 Bone marrow studies
 MIBG contrast scan can be used and combined with iodine as a contrast and may be used diagnostically and therapeutically as it is a NE
analogue.
Relevant Investigations
Staging
Overview of Management

Management can be medical or surgical:

1. Medical: chemotherapy and radiation
2. Surgical: surgical resection
Complications of the two may be tumor lysis syndrome allowing the patient to present with hyperkalemia,
hyperuricemia, and hypercalcemia. Also, immunosuppression would be significant. If the patient had resection,
intussception.
Prognosis

According to the INRGSS, children of any age with the localized disease and children under the age of 1 tend to
respond well to treatment. However, if seen in adolescence or adulthood, this has a prolonged course leading to a
poor prognosis. There may be a two year survival rate at this point.