Neuroblastoma Flashcards
(30 cards)
What is neuroblastoma?
A malignant neoplasm arising from neural crest cells of the sympathetic nervous system. It is the most common extracranial solid tumor in children.
What is the typical age of diagnosis for neuroblastoma?
The median age is 22 months, with 30% of cases in the first year of life.
What are the most common locations for neuroblastoma?
Abdomen (65%), chest (20%), neck (5%), pelvis (5%).
How might a neuroblastoma in the abdomen present?
As a large, nontender abdominal mass.
How might a neuroblastoma in the chest present?
Discovered during a routine chest radiograph for unrelated respiratory symptoms.
What symptoms can a pelvic neuroblastoma cause?
Constipation or bladder dysfunction.
What is Horner syndrome?
A condition that can be caused by a neuroblastoma in the neck due to impingement on sympathetic ganglia.
What are the signs of spinal column extension of a neuroblastoma?
Significant neurological deficits, potentially leading to paralysis.
What are common symptoms of bone marrow invasion in neuroblastoma?
Anemia, bruising, weakness, bone pain, swelling, limp, or pathological fractures.
What is ‘raccoon eyes’ in relation to neuroblastoma?
Periorbital swelling and proptosis that indicates involvement of the orbits.
What is the ‘blueberry muffin syndrome’?
The presence of blue subcutaneous nodules that represent tumor dissemination to the skin.
What are some paraneoplastic syndromes associated with neuroblastoma?
Intractable diarrhea, encephalomyelitis, neuropathy, and opsoclonus-myoclonus syndrome.
What genetic factors are associated with neuroblastoma?
Whole chromosome gains (hyperdiploidy) for a better prognosis, and segmental chromosomal aberrations such as MYCN amplification or deletions, associated with worse outcomes.
What is the significance of the MYCN oncogene in neuroblastoma?
MYCN amplification (at chromosome 2p24) is found in 25% of cases and is associated with high-risk neuroblastoma.
What are the diagnostic urine tests for neuroblastoma?
Measurement of urine catecholamines and their metabolites (e.g., dopamine, vanillylmandelic acid, homovanillic acid).
What are some non-specific tumor biomarkers associated with neuroblastoma?
Elevated levels of lactate dehydrogenase (>1500 U/mL), ferritin (>142 ng/mL), and neuron-specific enolase (>100 ng/mL).
What imaging techniques are used to diagnose neuroblastoma?
CT scans (for calcifications), MRI (for spinal cord extension), and 131I-metaiodobenzylguanidine (MIBG) scan (for metastases).
How is neuroblastoma risk stratified?
By the Children’s Oncology Group into low, intermediate, or high-risk categories, based on age, INSS stage, histopathology, DNA index, and MYCN amplification.
What is the typical initial treatment for localized neuroblastoma tumors?
Surgical resection.
What is the typical initial treatment for advanced-stage neuroblastoma?
An incisional biopsy for tumor biology studies, followed by chemotherapy.
What are the goals of surgical debulking for neuroblastoma?
To achieve more than 90% resection after induction therapy.
What are the main chemotherapy drugs used in neuroblastoma?
Cyclophosphamide, doxorubicin, cisplatin, carboplatin, etoposide, and vincristine.
What is a common treatment for high-risk neuroblastoma?
Autologous hematopoietic stem cell transplantation and anti-GD2 immunotherapy.
What is the role of radiation therapy in neuroblastoma treatment?
For local and metastatic control, but it’s contraindicated for intraspinal tumors.
