Neuroblastoma

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Neuroblastoma is a malignant tumour of the adrenal gland.

Olfactory neuroblastoma is dealt with separately.

General

Epidemiology:

  • Usually paediatric population.

Laboratory findings:

  • Increased urine homovanillic acid.

Imaging:

  • mIBG uptake (>90% neuroblastomas)


Predictors of a poor prognosis:[1]

  • High mitotic-karyorrhectic index.
  • Lack of schwannian stroma.
  • >18 months.
  • Near ploidy.
  • N-MYC amplification.
  • 1p deletion /imbalance
  • Lymph node spread.
  • Distant spread.

Classification:

Gross

Microscopic

Features:[4]

  • Small round blue cells separated by thin (pink) fibrous septa.
  • Homer-Wright rosettes.
    • Rosette with a small (~100 micrometers - diameter) meshwork of fibers (neuropil) at the centre.[5]
  • Neuropil-like stroma = paucicellular stroma with a cotton candy-like appearance; see comparison below.
    • >50% neuropil-like stroma -- otherwise it's a ganglioneurona or ganglioblastoma.

Notes:

  • The fibrous septa are especially useful for differentiation from lymphoma.

DDx:

Images

  • Neuroblastoma - medium power]]

  • Neuroblastoma - medium power (SKB)

  • Neuroblastoma - medium power]]

  • Neuroblastoma - vascular invasion - medium power]]

  • Neuroblastoma - medium power]]

  • Neuroblastoma - medium power]]

  • Neuroblastoma - high power (SKB)

  • Neuroblastoma - high power - blue cells arrayed around a core of fluffy pink neuropil (SKB)

  • Neuroblastoma - high power]]

  • Neuroblastoma - high power]]

Schwannian vs. neuropil

Feature Schwannian Neuropil
Cellularity high ~ spacing of cells < 30 µm low ~ spacing of cells > 100 µm
Fibrillary yes, long fine strands no
Associations ganglion cells neuroblasts
Cytoplasmic vacuolation yes ?

Classification/grading

Commonly grouped by the Shimada classification, which depends on the presence a number of things including:

  • Mitoses/karyorrhectic cells.
  • Molecular abnormalities.

International Neuroblastoma Risk Group Staging System (INRGSS)

  • L1: Locoregional tumor without Image-Definging Risk factors (IDRFs)
  • L2: Locoregional tumor with one or more IDRFs
  • M: Distant metastatic disease (except Ms)
  • Ms: INRG Stage L1 or L2 tumor with metastatic disease confined to skin and/or liver and/or bone marrow and age below 18 months.

IHC

  • PGP 9.5 +ve.[7]
    • PGP = protein gene product.
  • NB-84 +ve.[8]
    • More sensitive that synaptophysin.
  • Synaptophysin +ve.
  • CD99 -ve.

EM

Distinctive EM appearance:[9]

  • Dendritic processes with longitudinally oriented microtubules.
  • Membrane bound electron-dense granules (contain catecholamines).
  • Desmosomes
    • Not seen in EWS, RMS, lymphomas.
  • Membrane densities.

Pertinent negative:[9]

  • No glycogen.

See also

References

  1. ↑ Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 254. ISBN 978-1416054542.
  2. ↑ Shimada H, Ambros IM, Dehner LP, Hata J, Joshi VV, Roald B (July 1999). "Terminology and morphologic criteria of neuroblastic tumors: recommendations by the International Neuroblastoma Pathology Committee". Cancer 86 (2): 349–63. PMID 10421272.
  3. ↑ Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 253. ISBN 978-1416054542.
  4. ↑ Chung EM, Murphey MD, Specht CS, Cube R, Smirniotopoulos JG (2008). "From the Archives of the AFIP. Pediatric orbit tumors and tumorlike lesions: osseous lesions of the orbit". Radiographics 28 (4): 1193–214. doi:10.1148/rg.284085013. PMID 18635637.
  5. ↑ Wippold FJ, Perry A (March 2006). "Neuropathology for the neuroradiologist: rosettes and pseudorosettes". AJNR Am J Neuroradiol 27 (3): 488–92. PMID 16551982.
  6. ↑ URL: http://radiographics.rsna.org/content/28/4/1193.full. Accessed on: 12 January 2011.
  7. ↑ Ootsuka, S.; Asami, S.; Sasaki, T.; Yoshida, Y.; Nemoto, N.; Shichino, H.; Chin, M.; Mugishima, H. et al. (Jun 2008). "Useful markers for detecting minimal residual disease in cases of neuroblastoma.". Biol Pharm Bull 31 (6): 1071-4. PMID 18520032.
  8. ↑ Miettinen, M.; Chatten, J.; Paetau, A.; Stevenson, A. (Mar 1998). "Monoclonal antibody NB84 in the differential diagnosis of neuroblastoma and other small round cell tumors.". Am J Surg Pathol 22 (3): 327-32. PMID 9500774.
  9. ↑ 9.0 9.1 Mackay, B.; Masse, SR.; King, OY.; Butler, J. (Dec 1975). "Diagnosis of neuroblastoma by electron microscopy of bone marrow aspirates.". Pediatrics 56 (6): 1045-9. PMID 1196755.
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