Neuropathology tumours

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The article covers tumours in neuropathology. Tumours are a large part of neuropathology. Cytopathology of CNS tumours is dealt with in the article CNS cytopathology.

The article also includes peripheral nerve sheath tumours.

Brain tumours

Adult

Four most common types of brain tumours:[1]

  1. Metastatic brain tumours (barely edges out primary tumours)
  2. Glioblastoma aka glioblastoma multiforme.
  3. Anaplastic (malignant) astrocytoma.
  4. Meningioma.

Children

  1. Astrocytoma.
  2. Medulloblastoma.
  3. Ependymoma.

Location (most common)

Certain tumours like to hang-out at certain places:[2]

  • Cerebrum:
    • Cortical based - oligodendroglioma.
    • Grey-white junction - metastases.
    • White matter - astrocytoma, glioblastoma.
    • Periventricular - CNS lymphoma.
    • Cystic - ganglioglioma, pilocytic astrocytoma, pleomorphic xanthoastrocytoma.
  • Cerebellum:
    • Midline/central - medulloblastoma.
    • Cystic lesion - pilocytic astrocytoma (younger individual), hemangioblastoma (older individual).
    • Solid lesion (older individual) - metastasis.
  • Spinal cord:
    • Ependymoma, glioblastoma.
    • Filum terminale - myxopapillary ependymoma, paraganglioma.

Filum terminale

  • Filum terminale = bottom end of the spinal cord - has a limited differential.

DDx:[3]

  • Meningioma.
  • Myxopapillary ependymoma.
  • Neurofibroma.
  • Schwannoma.
  • Paraganglioma.

Cerebellopontine angle

DDx:[4]

  • Schwannoma.
  • Meningioma.
  • Dermoid cyst/epidermoid cyst.
  • Ependymoma.
  • Choroid plexus papilloma.

Primary vs. secondary

Glial tumours:

  • Cytoplasmic processes - key feature.
    • Best seen at highest magnification - usu. ~1 micrometer.
    • Processes may branch.
  • Ill-defined border/blend with the surrounding brain/.

Astrocytomas

Overview

  • Pilocytic astrocytomas (WHO Grade I).
  • Dysembryoplastic neuroepithelial tumour (DNT), (WHO Grade I).
  • Low-grade (diffuse) astrocytomas (Grade II).
  • Anaplastic astrocytomas (Grade III).
  • Glioblastoma (Grade IV).

Microscopic

Features:[5][6]

  • Glial processes - key feature.
    • Thin stringy cytoplasmic processes - best seen at high power in less cellular areas.

Images:

Notes:

  • Glial vs. non-glial tumours:
    • Glial: "blends into brain"/gradual transition to non-tumour brain.
    • Non-glial: no glial processes.

Grading

At least grade II:

  • Nuclear pleomorphism.

At least grade III:

  • Mitotic figures.

At least grade IV:

  • Microvascular proliferation or necrosis with pseudopalisading tumour cells.
    • Pseudopalisading tumour cells = high tumour cell density adjacent to regions of necrosis; palisade = a fence of pales forming a defense barrier or fortification.

Glioblastoma IHC

  • GFAP - should stain cytoplasm of tumour cells and the perikaryon (nuclear membrane).
  • Ki-67.
  • p53.
  • IDH1.
    • +ve in tumours that arose from low-grade gliomas.[7]

Notes:

  • IDH1 and IDH2 mutations - better survival.[8]

Pilocytic astrocytoma

General

  • Low-grade astrocytoma.
  • Classically in the cerebellum in children.
  • The optic glioma associated with neurofibromatosis 1.

Gross

  • Do NOT smear.

Microscopic

Features:

  • Rosenthal fibres - key feature.

Images:

DDx (of Rosenthal fibers):[9]

  • Chronic reactive gliosis.
  • Subependymoma.
  • Ganglioma.
  • Alexander's disease (rare leukodystrophy).

Oligodendroglioma

General

  • Arise from oligodendrocytes.

Usual location:

  • Fourth ventricle.
  • Intramedullary spinal cord.

Prognosis by flavours (average survival):[10]

  • WHO grade II: 10-15 years.
  • WHO grade III: 3-5 years.

Microscopic

Features:

  • Highly cellular lesion composed of:
    • Cells resembling fried eggs (oligodendrocytes) with:
      • Round nucleus - key feature.
      • Distinct cell borders.
      • Moderate-to-marked nuclear atypia.
      • Clear cytoplasm - useful feature (if present).
        • Some oligodendrogliomas have eosinophilic cytoplasm with focal perinuclear clearing.
    • Acutely branched capillary sized vessels - "chicken-wire" like appearance.
      • Abundant, delicate appearing; may vaguely resemble a paraganglioma at low power.
  • Calcifications - important feature.[11]

Images:

Notes:

  • Few neural tumours have round nuclei - DDx:
    • Oligodendroglioma.
    • Lymphoma.
    • Clear cell variant of ependymoma.
    • Germ cell tumour (dysgerminoma/seminoma).

Histologic grading

Come in two flavours:

  1. WHO grade II.
    • This is most oligodendrogliomas.
  2. WHO grade III.
    • Features for calling high grade:[10]
      • Endothelial hypertrophy.
        • Plump/large endothelial cells.
      • Necrosis.
      • High mitotic rate (6 mitoses/10 HPF for whatever "HPF" means, see HPFitis).

IHC

Features:

  • GFAP +ve.
  • EMA +ve.

Molecular pathology

Losses of 1p and 19q both helps with diagnosis and is prognostic:[12]

  • Greater chemosensitivity
  • Better prognosis.

Peripheral nerve sheath tumours

A classification:[13]

  • Benign:
    • Schwannoma.
    • Neurofibroma.
    • Perineurioma.
    • Traumatic neuroma.
  • Malignant:
    • Malignant peripheral nerve sheath tumour (MPNST).

Meningioma

Main article: Meningioma

General

  • Very common.
  • May be part of a syndrome.

Microscopic

Features (memory device WTC):

  • Whorled appearance - key feature.
  • Thick-walled blood vessels, usually prominent.
  • Calcification.

Grading: see meningioma.

Schwannoma

General

  • Tumour of tissue surrounding a nerve.
    • Axons adjacent to the tumour are normal... but may be compressed.

Microscopic

Features:[13]

  • Antoni tissue (type A and type B).
  • Verocay bodies - paucinuclear area surrounded by nuclei.

Antoni A

  • Cellular.
  • 'Fibrillary, polar, elongated'.

Comment: May look somewhat like scattered matchsticks.

Antoni B

  • Loose microcystic tissue.
  • Adjacent to Antoni A.

Micrographs:

Neurofibroma

Microscopic

General:[13]

  • Composed of Schwann cells, axons, fibrous material.

Appearance/morphology:[13]

  • Plexiform growth pattern - "bag of worms".

Ganglioneuroma

General

  • AKA ganglioma.[14]
  • May be retroperitoneal.

Microscopic

Features:

  • Ganglion cells - key feature.
    • Large cells with large nucleus.
      • Prominent nucleolus.
  • Disordered fibrinous-like material.
  • Eosinophilic granular bodies.[15]

Images:

See: Adrenal gland.

Ependymoma

General

  • Called the forgotten glial tumour.

Comes in two flavours:

  1. Ependymoma (not otherwise specified).
  2. Myxopapillary ependymoma.
    • Classically at filum terminale.

Microscopy

Classic ependymoma

Features:

  • Cells have a "tadpole-like" morphology.
    • May also be described as ice cream cone-shaped.[16]
  • Rosettes - cells arranged in a pseudoglandular fashion.
  • "Nucleus free zones" - cells arranged around a blood vessel (perivascular pseudorosettes); nuclei of cells distant from the blood vessel, i.e. a rim of cytoplasm (from tumour cells) surrounds the blood vessel.

Perivascular pseudorosettes = (tumour) cells arranged around a blood vessel; nuclei of cells distant from the blood vessel, i.e. rim of cytoplasm (from tumour cells) surround blood vessel (nucleus-free zone)

    • The nucleus free zone is composed of tumour cell cytoplasm that is adjacent to an unseen blood vessel.
  • Nuclear feature monotonous, i.e. "boring".[17]
    • There is little variation in size, shape and staining.

Images:

DDx (classic ependymoma):

  • Subependymoma.

Myxopapillary ependymoma

Features:

  • Perivascular pseudorosettes:
    • Myxoid material surround blood vessels.
      • Myxoid material surrounded by tumour cells.

Images:

Choroid plexus papilloma

Microscopy

Features:

  • Papillae.
  • Psammoma bodies.

Image:

Chordoma

General

  • Location: usually sacrum or clivus.

Microscopic

Features:[18]

  • Architecture: islands of cells surrounded by fibrous tissue.
    • Also described as "lobulated" architecture; may not be apparent.
  • Myxoid background - grey extracellular material, variable amount present.
  • Mixed cell population:
    1. Abundant eosinophilic cytoplasm.
    2. Physaliphorous cells or bubble cells - key feature.
      • Have a very large clear bubble with a sharp border; bubble does not compress nucleus - nucleus may be in bubble.

Image(s):

IHC

Features:

  • S100 +ve.
  • CK +ve.
  • Brachyury +ve.
    • Protein important for axial development, affects notochord development.[19]
    • Brachyury literally means short tail.[20]

Hemangioblastoma

General

Microscopic

Features:[21]

  • Vascular.
  • Polygonal stromal cells with:
    • Hyperchromatic nuclei.
    • Vacuolar cytoplasm.

Images:

Medulloblastoma

General

  • Paediatric population. (???)

Microscopic

Features:[22]

  • Homer-Wright rosettes.

Image:

See also

References

  1. ↑ http://neurosurgery.mgh.harvard.edu/abta/primer.htm
  2. ↑ URL: http://www.msdlatinamerica.com/ebooks/DiagnosticNeuropathologySmears/files/4ce563fb7e8e48fc9ed8b42e296a7747.gif and http://www.msdlatinamerica.com/ebooks/DiagnosticNeuropathologySmears/sid117213.html. Accessed on: 2 November 2010.
  3. ↑ JLK. 31 May 2010.
  4. ↑ R. Kiehl. 8 November 2010.
  5. ↑ Rong Y, Durden DL, Van Meir EG, Brat DJ (June 2006). "'Pseudopalisading' necrosis in glioblastoma: a familiar morphologic feature that links vascular pathology, hypoxia, and angiogenesis". J. Neuropathol. Exp. Neurol. 65 (6): 529–39. PMID 16783163.
  6. ↑ http://dictionary.reference.com/browse/palisading
  7. ↑ PMID 19228619.
  8. ↑ PMID 20975057.
  9. ↑ MUN. 9 Mar 2009.
  10. ↑ 10.0 10.1 Perry, Arie; Brat, Daniel J. (2010). Practical Surgical Neuropathology: A Diagnostic Approach: A Volume in the Pattern Recognition series (1st ed.). Churchill Livingstone. pp. 98. ISBN 978-0443069826.
  11. ↑ URL: http://www.emedicine.com/radio/topic481.htm.
  12. ↑ Fontaine D, Vandenbos F, Lebrun C, Paquis V, Frenay M (2008). "[Diagnostic and prognostic values of 1p and 19q deletions in adult gliomas: critical review of the literature and implications in daily clinical practice]" (in French). Rev. Neurol. (Paris) 164 (6-7): 595–604. doi:10.1016/j.neurol.2008.04.002. PMID 18565359.
  13. ↑ 13.0 13.1 13.2 13.3 Wippold FJ, Lubner M, Perrin RJ, Lämmle M, Perry A (October 2007). "Neuropathology for the neuroradiologist: Antoni A and Antoni B tissue patterns". AJNR Am J Neuroradiol 28 (9): 1633–8. doi:10.3174/ajnr.A0682. PMID 17893219. http://www.ajnr.org/cgi/reprint/28/9/1633.
  14. ↑ URL: http://medical-dictionary.thefreedictionary.com/ganglioma. Accessed on: 8 November 2010.
  15. ↑ R. Kiehl. 8 November 2010.
  16. ↑ http://www.pathology.vcu.edu/WirSelfInst/tumor-2.html
  17. ↑ MUN. 6 Oct 2009.
  18. ↑ Tadrous, Paul.J. Diagnostic Criteria Handbook in Histopathology: A Surgical Pathology Vade Mecum (1st ed.). Wiley. pp. 184. ISBN 978-0470519035.
  19. ↑ URL:http://www.ncbi.nlm.nih.gov/omim/601397. Accessed on: 18 May 2010.
  20. ↑ URL: http://www.jstor.org/pss/86845. Accessed on: 18 May 2010.
  21. ↑ URL: http://emedicine.medscape.com/article/340994-media. Accessed on: 23 June 2010.
  22. ↑ URL: http://moon.ouhsc.edu/kfung/jty1/neurotest/Q93-Ans.htm. Accessed on: 26 October 2010.

External links

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