Feature |
Description |
Origin |
Neural crest cells (sympathetic |
Common |
Adrenal medulla (most common), |
Age |
Almost exclusively in children, most <5 years |
Clinical |
- Abdominal mass (often crosses midline) - Weight loss, fatigue - Bone pain (metastases) - Periorbital ecchymoses (raccoon eyes) - Opsoclonus-myoclonus syndrome |
Metastasis |
Bone, liver, skin, bone marrow |
Paraneoplastic |
- Opsoclonus-myoclonus - Increased catecholamines (increase |
Imaging |
CT/MRI - large heterogeneous mass with calcifications |
Diagnosis |
Biopsy + Elevated urinary VMA/HVA |
Histology |
- Small round blue cells - Homer Wright pseudo rosettes |
Markers |
- NSE (Neuron Specific Enolase) - N MYC amplification (poor prognosis) - Biomarkesrs: Bombesin, Synaptophysin, |
Homer Wright pseudo rosettes seen in: (Centre is filled, not empty)
- Medulloblastoma
- Ewing sarcoma
- Neuroblastoma (Adrenal medulla tumor)
- Retinoblastoma: White pupillary reflex, Strabismus
Flexner Wintersteiner true rosettes seen in: (Centre is empty)
- Retinoblastoma: White pupillary reflex, Strabismus