MCQ – 88704

A 4-year-old girl is brought to the pediatric clinic by her mother who reports that her daughter has decreased appetite, lethargy, and an enlarging belly. Physical examination reveals a large, firm, irregular, nontender mass in the child’s abdomen. A CT-guided biopsy reveals neoplastic “small blue cells.” The child’s malignant neoplasm is removed and the surgical specimen is shown in the image. Which of the following laboratory tests would be useful in monitoring this patient for recurrence of disease?

Correct Answer � D Explanation

Answer : D Urinary vanillylmandelic acid

Neuroblastoma is a malignant tumor of neural crest origin that is composed of neoplastic neuroblasts, and originates in the adrenal medulla or sympathetic ganglia (note the suprarenal location of the tumor).

The neuroblast is derived from primitive sympathogonia and represents an intermediate stage in the development of the sympathetic ganglion neurons. 

On histologic examination, pseudorosettes, featuring tumor cells clustered radially around small vessels, are present.

Tumor cells, like their more mature descendants in the adrenal medulla, may secrete catecholamines. 

These compounds are metabolized and excreted as urinary vanillylmandelic acid in the urine.

Neuroblastoma is one of the most important malignant tumors of childhood, accounting for up to 10% of all childhood cancers and 15% of cancer deaths among children. 

The overall incidence is 1 in 7,000. The peak incidence is in the first 3 years of life. The other choices do not serve as markers for neuroblastoma or other childhood malignancies.