Neuroblastoma

Question 1

What is the most common malignancy diagnosed in infancy?

Answer 1

Neuroblastoma makes up 8-10% of all childhood cancers and is the most common malignancy diagnosed during infancy.

Question 2

From where do most neuroblastomas arise?

Answer 2

40% of neuroblastomas arise in the abdomen within the adrenal medulla, 30% arise from the nonadrenal abdomen, about 20% occur in the paravertebral ganglia of the chest or neck.

Question 3

What is the typical presentation of neuroblastoma?

Answer 3

Identification of a nontender abdominal mass, typically retroperitoneal.

Question 4

What symptoms does VIP cause in neuroblastoma?

Answer 4

Intractable secretory diarrhea and abdominal distention due to secretion of vasoactive intestinal peptide.

Question 5

The identification of either of what two syndromes should prompt workup for neuroblastoma?

Answer 5

VIP syndrome and Opsoclonus-myoclonus-ataxia syndrome

Question 6

Describe opsoclonus-myoclonus-ataxia syndrome.

Answer 6

Rapid and chaotic eye movements, ataxia, and myoclonus (dancing eyes-dancing feet syndrome). It occurs in ~5% of newly diagnosed neuroblastoma patients.

Question 7

How do you diagnose neuroblastoma?

Answer 7

Diagnosis requires biopsy with histologic evidence of neural origin of the tumor, or, if using bone marrow to make the diagnosis, compatible “clumps” of cells.

Question 8

What tumor markers are useful in both diagnosis and therapeutic/post-therapeutic monitoring of neuroblastoma?

Answer 8

Urinary levels of homovanillic acid (HVA) and vanillylmandelic acid (VMA).

Question 9

What tests are usually performed as part of the workup for neuroblastoma?

Answer 9

Bilateral bone marrow aspirate and biopsy, plain radiographs, bone scintigraphy, CT, MRI, MIBG scintigraphy, and urine HVA and VMA levels.

Question 10

At what age of diagnosis do children have the best prognosis in neuroblastoma?

Answer 10

Best prognosis occurs when diagnosis is made at <18 months of age

Question 11

What is the treatment for low-risk neuroblastoma?

Answer 11

Surgery alone

Question 12

What is the treatment for intermediate-risk neuroblastoma?

Answer 12

Surgery and chemotherapy

Question 13

What is the treatment for high-risk neuroblastoma?

Answer 13

Surgery, standard chemotherapy, high-dose chemotherapy with autologous stem cell rescue, radiation, cis-retinoic acid, and immune-modulating therapy. Even with aggressive therapy, outcomes in these children are poor.

Question 14

What chemotherapeutic drugs are commonly used to treat neuroblastoma?

Answer 14

Cyclophosphamide, ifosfamide, cisplatin, carboplatin, doxorubicin, and etopaside.