10.6.4.5 Liver

In infants, marked hepatomegaly, pathologists speak of “pepper type” liver.

10.6.4.6 Skin

• Subcutaneous nodules of blue color which become reddish and then white owing to vasoconstriction from release of catecholamines after palpation; these are most commonly observed in neonates or infants with disseminated neuroblastoma

10.6.4.7 Bone

• Bone pain, sometimes as one of the first signs

• Involvement mainly in the skull and long bones

• On X-rays, seen as lytic defects with irregular margins and periosteal reactions

10.6.4.8 Bone Marrow

•Infiltration in more than 50% of patients

•Peripheral thrombocytosis may indicate early stage of bone marrow infiltration

•Peripheral thrombocytopenia and/or anemia indicate advanced stage of bone marrow infiltration

10.7Metastatic Spread

•Lymphatic and/or hematogenous spread

•Often initially present in:

–Forty to fifty percent of children less than 1 year of age

–Seventy percent of children more than 1 year of age

•In children with local neuroblastoma, 35% have involvement of lymph nodes

•Metastatic spread mostly in bone marrow, bone, liver, and/or skin, rarely in brain, skull, orbit with proptosis, spinal cord, heart, or lung with associated symptoms, e.g., bone pain, pancytopenia

10.8Laboratory Findings

10.8.1 Urinary Catecholamine Metabolites (Tyrosine Metabolism)

•High levels of vanillylmandelic acid (VMA) in 95%, homovanillic acid (HVA) in 90%, and 3-methoxy-4-hydroxyphenylglycol (MHPG) in 97% of patients

•Other metabolites of catecholamine metabolism for differentiation of pheochromocytoma, olfactory neuroblastoma, and melanoma

•Spot tests with some false-positive and false-negative results

•Urinary catecholamine metabolite analyses useful: follow-up tumor marker