Neuroblastoma
With Dr. Dan von Allman & Dr. Erika Neumann & Dr. Tony Sandler · hosted by Dr. Ray Hanky & Dr. Todd Ponsky · StayCurrentMD
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Neuroblastoma 26 items
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Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
Podcast
Neuroblastoma
56 min · Published Aug 2019
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What the experts said
Adrenal hemorrhage is the most common differential diagnosis for prenatal suprarenal mass, more common with history of fetal stress
Other differential diagnoses for suprarenal mass include neuroblastoma, pulmonary sequestration below the diaphragm, and misdiagnosed renal anomaly
Familial neuroblastoma occurs in about 1% of patients
For prenatal suprarenal mass, first postnatal study should be ultrasound of the abdomen
CT scan or MRI not needed for 3 cm lesion unless urine catecholamines are elevated
MIBG scan is the next step if catecholamines are elevated
Radiologists are quite good at identifying adrenal hemorrhage on ultrasound
In perinatal phase, most common metastatic sites are liver, bone, skin, and lymph nodes
Nocktern study data supports observation of prenatal neuroblastoma with careful ultrasound surveillance
Of 84 observed patients in Nocktern study, 16 (approximately 20%) underwent resection for growth or family preference
Nocktern study showed approximately 98% event-free survival and 100% overall survival in observed prenatal neuroblastoma
First-year surveillance protocol: ultrasound and catecholamines at birth, 3 weeks, 6 weeks, 12 weeks, then spaced out to one year
After one year, surveillance becomes every six months, then yearly
Case report: child with observed prenatal adrenal mass that resolved presented at age 3 with widely metastatic high-risk neuroblastoma
5 centimeters is used as size cutoff for surgical intervention in observed prenatal masses
Volume increase of more than 50% is criterion for considering surgery
50% increase in VMA or HVA prompts consideration of surgery
Laparoscopic approach is reasonable for masses less than 6 centimeters
Lymph node status in neuroblastoma is not as important for therapy changes as in Wilms tumor
Biology of neuroblastoma is more important than lymph node status for treatment decisions
Stage MS (formerly 4S) with skin lesions and liver mets still tends to have good biology
Primary concern in stage MS with liver involvement is mass effect causing respiratory compromise
Stage MS without distress can be treated with aggressive observation
Once respiratory compromise begins, treatment options include chemotherapy, radiation, or emergent decompressive laparotomy
Classic findings of stage MS (high catecholamines, blue blebs on skin, liver metastasis, adrenal mass) may not require biopsy
Liver biopsy in newborns is difficult because bleeding is hard to control
If NMYC is amplified in stage MS, staging changes from MS to M
VIP secretion can cause severe diarrhea in neuroblastoma
Initial workup for abdominal mass includes ultrasound to determine solid vs cystic, then CT with PO and IV contrast if solid
Ultrasound is important for Wilms tumor to assess venous extension
Large mass encasing aorta and celiac axis with microcalcifications represents L2 INRG classification
Complete staging workup includes bone marrow biopsy, MIBG scan, chest CT to rule out metastasis, and head CT if clinical symptoms present
10% of neuroblastomas are not MIBG avid
PET scan may detect metastases in MIBG-negative neuroblastomas
PET scan is not part of routine initial diagnostic workup but may be used for MIBG-negative soft tissue areas to distinguish recurrence from scar
Open retroperitoneal biopsy provides adequate tissue size for pathology and biology studies
Transperitoneal laparoscopic biopsy may not allow adequate bleeding control for large tumors
Multiple percutaneous biopsies may not provide adequate tissue for biology studies
NMYC amplification can be obtained from bone marrow, but additional biology studies require tumor tissue
Biology studies beyond NMYC include ALK mutation and ploidy status