Neuroblastoma

Feature

Description

Origin

Neural crest cells (sympathetic
nervous system)

Common
Site

Adrenal medulla (most common),
followed by paraspinal sympathetic chain

Age
Group

Almost exclusively in children, most <5 years

Clinical
Features

- 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
Syndrome

- Opsoclonus-myoclonus

- Increased catecholamines (increase
VMA/HVA in urine)

Imaging

CT/MRI - large heterogeneous mass with

calcifications

Diagnosis

Biopsy + Elevated urinary VMA/HVA
(catecholamine metabolites)

Histology

- Small round blue cells

- Homer Wright pseudo rosettes

Markers

- NSE (Neuron Specific Enolase)

- N MYC amplification (poor prognosis)

- Biomarkesrs: Bombesin, Synaptophysin,
Chromogranin

 

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 

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