Neuroblastoma936-8

Case Records

Of the

Massachusetts General Hospital

Case 27-1995

New England Journal of Medicine

1995;333:579-586

Figure 1. Saggital ultrasound image of the left upper quadrant of the abdomen, showing a heterogenous left adrenal mass.

Figure 2. Contrast-enhanced abdominal CT scan showing a 3.5 cm, heterogenous left adrenal mass.

Figure 3. Sectioned surface of the left adrenal gland showing the tumor surrounded by a thin yellow rim of residual adrenocortical tissue.

Figure 4. Nodular tumor and rim of the adrenal gland (hematoxylin and eosin, x4)

Figure 5. Lymphoid tissue within tumor nodules (hematoxylin and eosin, x48).

Figure 6. Round neuroblasts with a delicate fibrillary background (hematoxylin and eosin, x90).

Figure 7. Ganglion cells mixed with lymphocytes (hematoxylin and eosin, x90).

Paraneoplastic (Neuroblastoma) Opsocolonus

Opsoclonus herald sign 2-3%

Mean age at onset 16 years

Youngest 4 months of age

Older than 2 years in only 13%

Mediastinal tumor in 49-61%

Tumor found within 3 months in 60%

Rarely associated with elevated catecholamines (VMA, HVA metabolites)

Anti-neurofilament protein antibodies (M V 210 K) ± in sera

Paraneoplastic (Neuroblastoma) Opsocolonus

Survival rate 90% two-year c.f. 30-34% other neuroblastoma cases

Tumor removal may decrease, have no effect or exacerbate opsoclonus

Spontaneous resolution may take months or years

Paraneoplastic (Neuroblastoma) Opsocolonus

http://www.library.med.utah.edu/NOVEL