Neuroblastoma

Epidemiology
Most common cancer in infants
700 cases per year
Median age at diagnosis is 2 years
Screening programs have been tested (famously in Japan) without affecting the mortality rate
No screening is currently offered

Prognostic factors
Unfavorable
-n-myc amplification
-unfavorable Shimada classification
-diploid DNA
-LOH at 1p
-Age older than one year
-Higher stage

Clinical
Most common site adrenal glands, then paraspinal
70% have mets at diagnosis
Skin mets: "Blueberry muffin" sign; blanch when pressed
85% of masses are calcified

Tumor markers: urinary and serum catecholamines (VMA, HVA)

Paraneoplastic syndromes: opsoclonus/myoclonus syndrome, fever, weight loss, hyper-VIP-ism

Staging: should include MIBG scan, bone marrow biopsy

Therapy
Low risk = stage I - II or IVS with favorable histology, normal n-myc, aneuploid
-Surgery only for stage I (gross total resection)
-Surgery + chemo for stage IIA (subtotal resection, node negative)
-Observation for IVS patients

Intermediate risk = stage IIA - IIB with any histology; III (unresectable) with favorable histology, infants with stage IV
-Induction chemo
-Surgery
-RT (24Gy) for residual disease)

High risk = Older than one year with mets; any age or stage with unfavorable pathology
-High intensity chemo with BMT
-Adjuvant cis-retinoic acid
-RT to primary and sites of bulky disease (24-30Gy)