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Neuroblastoma
Etiology

Although most cases seem to occur sporatically, there is strong evidence for genetic factors.
Pathogenesis

Neuroblastomas are believed to arise from primitive cells of neural crest origin.,
Epidemiology

Neuroblastoma is one of the more common childhood malignancies.
It accounts for 15% of childhood cancer deaths.
35% of neuroblastoma cases are diagnosed in the first year of life.
20% of neuroblastoma cases are diagnosed in the second year of life.
90% of cases are diagnosed under the age of five.
General Gross Description

Tumors may be small to massive (1 kg).
Cut surface is usually soft, fleshy, and brain-like with variegated areas of necrosis and hemorrhage.
These tumors have a tendency to have calcifications which can be a helpful in radiologic studies.
•Examples:
Neuroblastoma encasing kidney
General Microscopic Description

Histologically, neuroblastoma is one of the so-called small blue round cell tumors of childhood.
The tumor is generally composed of small (lymphocyte-size) cells with round nuclei and scant cytoplasm.
Areas of differentiation to look for are spindled cell contour with cytoplasmic extensions, abundant perinuclear cytoplasm resembling ganglion cells, and circular arrangement of nuclei without lumenal definition (rosette formation).
Microcalcifications are also a histologic feature.
•Examples:
Clinical Correlation

Presents as a mass lesion.
In the region of the kidney such as this case, the most likely origin is the adrenal gland medulla.
Another common site of origin is the paravertebral sympathetic chain either thoracic or abdominal.
Some cases are discovered by metastatic lesions.
Some cases may regress or mature to ganglioneuromas either spontaneously or by chemotherapy.
References

Cotran RS, Kumar V, Robbins SL: Robbins Pathologic Basis of Disease. 5th ed. Philadelphia, W.B. Saunders, 1994, pp. 459-461.

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Neuroblastoma
Synopsis by: Harold Yamase M.D. (T71000M80006)[452]
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