Meningioma

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Meningioma a very common tumour in neuropathology.

Meningioma
Diagnosis in short

Meningioma. H&E stain.

LM whorled appearance, calcification - psammomatous, +/-nuclear pseudoinclusions
Subtypes Grade I (meningothelial, fibrous, transistional, psammomatous, angiomatous, microcystic, secretory, lymphoplasmacyte-rich, metaplastic), Grade II (invasive, clear cell, chordoid), Grade III (papillary, rhabdoid)
LM DDx schwannoma, solitary fibrous tumour, others
IHC EMA +ve, keratins usu. -ve, CD34 -ve/+ve, S-100 -ve (usu.), PR +ve (-ve in more aggressive ones)
Site see CNS tumours

Syndromes neurofibromatosis 2, nevoid basal cell carcinoma syndrome,Cowden syndrome

Clinical history +/-radiation
Prevalence common
Radiology extra-axial, intradural lesion
Prognosis usually benign, dependent on grade

General

Prevalence

  • Common.
  • May be caused by prior radiation.

Prognosis

  • Most are benign - usu. a good prognosis.
    • May be malignant - bad prognosis.

Genetics

Gross/Radiology

  • Extra-axial, intradural.
    • Can be extradural - very rare.[2]

Microscopic

Features (memory device WCN):

  • Whorled appearance - key feature.
  • Calcification, psammomatous (target-like appearance; (tight) onion skin).
  • +/-Nuclear pseudoinclusions - focal nuclear clearing with a sharp interface to unremarkable chromatin.

Notes:

  • May involute into benign sclerotic tissue.[3]
  • Thick-walled blood vessels -> think schwannoma.

DDx:

Images

www:

Morphologic subtypes

  • Many subtypes exist.[4]
  • The histologic subtypes generally don't have much prognostic significance.
    • Some subtypes are high grade by definition; also see histologic grading.

Grade I

Meningothelial meningioma
  • Most common.

Microscopic:

Fibrous meningioma
  • AKA fibroblastic meningioma.
  • Not collagen... but looks like it.
    • It is really laminin or fibronectin.
Transistional meningioma
  • Rare.
Psammomatous meningioma

Microscopic:

Angiomatous meningioma
  • AKA vascular.
  • May bleed like stink.
Microcystic meningioma

Microscopic:

  • Cystic appearance.
Secretory meningioma
  • Associated with brain edema; may have a worse outcome.

Microscopic:[5]

  • Eosinophilic intracytoplasmic inclusions that are CEA +ve and PAS +ve.

DDx:

Images:

Lymphoplasmacyte-rich meningioma

Microscopic:

  • Lymphocytes.
  • Plasma cells.

Images:

Metaplastic meningioma
  • Much talked about... but very rare.

Microscopic:

  • Cartilage or bone formation.

Grade II

Invasive meningioma
  • Invades the brain.

Images:

Clear cell meningioma

Epidemiology:

  • Usu. spinal cord.[7]

Microscopic:

  • Clear cells - contain glycogen (PAS +ve).

Images:

Chordoid meningioma
  • Chordoma-like.

Microscopic:

Image:

Grade III

Papillary meningioma

Microscopic:

  • True papillae.
Rhabdoid meningioma

Microscopic:

  • Rhabdoid appearance (abundant cytoplasm).
    • Cross-striations.

Images:

Histologic grading

Grading:[4]

  • Grade 1:
    • Low mitotic rate (< 4 mitoses/10 HPF - for whatever HPF means, see HPFitis).
    • Excludes clear cell, chordoid, papillary, and rhabdoid subtypes.
  • Grade 2 (either #1, #2 or #3):
    1. Brain-invasive meningioma.
      • Protrusion of meningioma into brain.
        • Meninogioma with entraped GFAP +ve tissue.
    2. Atypical meningioma (by histomorphology) - either A or B.
      • A. Intermediate mitotic rate (>= 4 mitoses/10 HPF - for whatever HPF means, see HPFitis.)
      • B. Three of the following five features:
        1. Sheeting architecture.
        2. High NC ratio clusters; clusters of "lymphocyte-like" cells.
        3. Hypercellularity.
        4. Macronucleoli.
        5. Necrosis not caused by treatment, e.g. radiation or embolization.
    3. Clear cell or chordoid subtype.
  • Grade 3 (either of the following):
    • High mitotic rate (>=20 mitoses/10 HPF - for whatever HPF means, see HPFitis.)
    • "Frank anaplasia"; marked nuclear atypia.
    • Papillary or rhabdoid subtype.

Notes:

  • Grade II soft criteria memory device HMNs: hypercellular, macronucleoli, NC ratio increased, necrosis, sheeting.

IHC

  • EMA +ve.[8]
  • Other CKs usually -ve.

DDx of meningioma & IHC[9]

Standard work-up (UHN)

  • Ki-67 >5-10% - predicts re-occurrence.[10]
  • PR (progesterone receptor) +ve in > 80% of meningiomas.[11]
    • Loss of PR staining predicts recurrence.
    • Strong association with tumour grade:[12]
      • Low WHO grade tumours usu. +ve.
      • High WHO grade tumours usu. -ve.

See also

References

  1. URL: http://moon.ouhsc.edu/kfung/jty1/neurotest/Q13-Ans.htm. Accessed on: 26 October 2010.
  2. URL: http://path.upmc.edu/cases/case702.html. Accessed on: 2 February 2012.
  3. URL: http://radiographics.rsna.org/content/23/3/785.long. Accessed on: 3 November 2010.
  4. 4.0 4.1 Perry, Arie; Brat, Daniel J. (2010). Practical Surgical Neuropathology: A Diagnostic Approach: A Volume in the Pattern Recognition series (1st ed.). Churchill Livingstone. pp. 194. ISBN 978-0443069826.
  5. URL: http://moon.ouhsc.edu/kfung/jty1/Com04/Com405-1-Diss.htm. Accessed on: 12 October 2011.
  6. URL: http://moon.ouhsc.edu/kfung/jty1/Com04/Com405-1-Diss.htm. Accessed on: 3 January 2012.
  7. Perry, Arie; Brat, Daniel J. (2010). Practical Surgical Neuropathology: A Diagnostic Approach: A Volume in the Pattern Recognition series (1st ed.). Churchill Livingstone. pp. 200. ISBN 978-0443069826.
  8. Perry, Arie; Brat, Daniel J. (2010). Practical Surgical Neuropathology: A Diagnostic Approach: A Volume in the Pattern Recognition series (1st ed.). Churchill Livingstone. pp. 13. ISBN 978-0443069826.
  9. Hahn HP, Bundock EA, Hornick JL (February 2006). "Immunohistochemical staining for claudin-1 can help distinguish meningiomas from histologic mimics". Am. J. Clin. Pathol. 125 (2): 203–8. doi:10.1309/G659-FVVB-MG7U-4RPQ. PMID 16393681. http://ajcp.ascpjournals.org/content/125/2/203.full.pdf.
  10. Croul, SE. 8 November 2010.
  11. Takei, H.; Buckleair, LW.; Powell, SZ. (Feb 2008). "Immunohistochemical expression of apoptosis regulating proteins and sex hormone receptors in meningiomas.". Neuropathology 28 (1): 62-8. doi:10.1111/j.1440-1789.2007.00852.x. PMID 18021195.
  12. Tao, Y.; Liang, G.; Li, Z.; Wang, Y.; Wu, A.; Wang, H.; Lu, Y.; Liu, Z. et al. (May 2012). "Clinical features and immunohistochemical expression levels of androgen, estrogen, progesterone and Ki-67 receptors in relationship with gross-total resected meningiomas relapse.". Br J Neurosurg. doi:10.3109/02688697.2012.685780. PMID 22616825.

External links