neuroblastoma Flashcards
What is neuroblastoma?
Neuroblastoma arises from primitive adrenergic neuroblasts of neural crest tissue in the sympathetic ganglion.
How common is neuroblastoma in children?
It is the 4th most common malignancy in children and the most common malignancy in children under 18 months.
What is the median age at diagnosis for neuroblastoma?
The median age at diagnosis is 22 months.
What is the clinical heterogeneity of neuroblastoma?
The spectrum of disease includes spontaneous maturation to rapid metastatic disease progression.
Where does neuroblastoma commonly arise?
Common locations include:
- Adrenal medulla (35%)
- Para-spinal ganglia (lower thoracic and abdominal) (30%)
- Posterior mediastinum (19%)
- Pelvic ganglia (2%)
- Cervical ganglia (1%)
What are common sites of metastases for neuroblastoma?
Metastases can occur in bone, bone marrow, liver, lymph nodes, and skin.
What are the constitutional symptoms of neuroblastoma?
Constitutional symptoms include anorexia, weight loss, malaise, and fever.
What is the most common presenting symptom of neuroblastoma?
Pain is the most common presenting symptom due to local spread or metastatic disease.
What are some other clinical manifestations of neuroblastoma?
Other signs include:
- Abdominal lump
- Respiratory compromise (especially in young infants with massive hepatomegaly)
- Horner’s syndrome
- Spinal cord compression
- Cytopenias (marrow involvement)
- Blueberry muffin sign (skin involvement)
- Bowel/bladder dysfunction (pelvic mass)
- Opsoclonus-myoclonus syndrome (truncal ataxia & cerebellar encephalopathy)
- Proptosis/ecchymoses (orbital mets)
What is the median age of presentation for neuroblastoma?
Approximately 1.5 years.
What is the median age of presentation for Wilms tumor?
Greater than 3 years.
How does neuroblastoma differ in midline crossing compared to Wilms tumor?
Neuroblastoma often crosses the midline, while Wilms tumor does not.
How do the appearances of patients with neuroblastoma and Wilms tumor differ?
Wilms patients appear healthy, while neuroblastoma patients appear sick.
What percentage of neuroblastoma cases exhibit calcifications?
85%.
What percentage of Wilms tumor cases exhibit calcifications?
5-10%.
How does metastasis to the lungs differ between neuroblastoma and Wilms tumor?
Neuroblastoma rarely metastasizes to the lungs, while Wilms tumor frequently does.
What is the origin of Wilms tumor?
Wilms tumor arises from and destroys the kidney.
What is the effect of neuroblastoma on the kidney?
Neuroblastoma displaces the kidney.
What routine tests are included in the laboratory evaluation for diagnosis?
Hemogram and biochemistry.
What urinary metabolites are evaluated in neuroblastoma diagnosis?
Vanillylmandelic acid (VMA) and Homovanillic acid (HVA).
What imaging techniques are used for neuroblastoma diagnosis?
X-ray, USG abdomen, and CE CT/MRI.
What is the purpose of a biopsy in neuroblastoma diagnosis?
Essential for tissue diagnosis, except when bone marrow and urinary catecholamine metabolites are diagnostic.
What is integral for management in neuroblastoma?
Tumor cytogenetics.
