Meningioma

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Meningioma a very common tumour in neuropathology.

Meningioma
Diagnosis in short

Meningioma. HPS 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, hemangiopericytoma, 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
Clinical history +/-radiation
Prevalence common
Radiology extra-axial, intradural lesion, dural tail sign (on MRI)
Prognosis usually benign, dependent on grade
Clin. DDx dependent on site - see CNS tumours
Treatment surgical removal

General

Prevalence

  • Most common primary brain tumour.[1]
  • May be caused by prior radiation.[2]

Prognosis

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

Genetics

Quick overview

Name Histologic criteria Subtypes Image
Classic, WHO I less then 4 mit/10 HPF and no atypia meningeothelial, fibroblastic, transitional, psammomatous, angiomatous, microcytsic, secretory, lymphoplasmacyte-rich, metaplastic
 
Atypical, WHO II brain invasion, 4 or more mit/10 HPF, or 3 of the following: necrosis, increased cellularity, high nuc:cyto ratio, nucleoli, sheeting chordoid, clear cell
 
Anaplastic, WHO III 20 or more mitoses/10 HPF, morphologiy similiar to carcinoma or sarcoma rhabdoid, papillary
 

Gross/Radiology

  • Extra-axial, intradural.
    • Can be extradural - very rare.[6]
  • Dural tail sign (DTS) on MRI.[7][8]
    • Enhancement of dura adjacent to the mass lesion - commonly seen (~70% of cases).[9]
    • May be subclassified radiologically - predictive of grading.[10]
  • +/-Hyperostosis.
    • Associated with invasion into the skull in ~20% of cases.[11]

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.[12]
  • Thick-walled blood vessels -> think schwannoma.

DDx:

Images

  • Meningioma - high mag. (WC)

  • Meningioma - intermed. mag. (WC)

  • Meningioma with brain invasion - intermed. mag. (WC)

  • Meningioma with brain invasion - high mag. (WC)

www:

Morphologic subtypes

  • Many subtypes exist.[13]
  • 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:

  • Syncytial, nuclear clearing (pseudoinclusions).
  • Whorls, Onion bulb formations.
  • Few psammoma bodies.

  • Syncytial appearance, whorl formations (WC/jensflorian)

  • Psammoma body (WC/Netha Hussain)

  • Onion bulb formation in smear (WC/jensflorian)

  • Meningioma HPS stain (WC/Nephron)

Fibrous meningioma
  • AKA fibroblastic meningioma.
  • Not collagen... but looks like it.
    • It is really laminin or fibronectin.
  • Spindle cells in parallel bundles.
  • Few to none whorl formations.

  • Fibrous meingioma (WC)

  • EMA staining (WC/marvin 101)

Transitional meningioma
  • AKA mixed.
  • Common.
  • Lobular and fasicular growth patters coexist.
  • Usu. a mixture of meningeothelial and fibromatous meningioma

  • Low power (WC/KGH)

  • Intermed magnification (WC/KGH)

  • Mitosis in a transitional meningioma (WC/jensflorian)

Psammomatous meningioma

Microscopic:

  • Psammoma bodies dominate over tumor cells.
    • Irregular calcifications (confluent psammoma bodies).
  • Usually found in spinal cord.

  • Numerous psammoma bodies (WC/jensflorian)

Angiomatous meningioma
  • AKA vascular.
  • May bleed like stink.
  • May show extensive edema.
  • Hyalinized vessels dominate over tumor cells.
  • Degenerative nuclear atypia.

DDx:

  • Vascular malformatons
  • Hemangioblastoma

  • Angiomatous meningioma (WC/jensflorian)

Microcystic meningioma

Microscopic:

  • Cystic appearance.
  • Increased cytologic pleomorphism of the elongated cells.

DDx:

  • Clear cell meningioma
  • Hemangioblastoma

  • Microcyts (WC/jensflorian)

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

Microscopic:[14]

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

Molecular:

  • Combined KLF4 K409Q and TRAF7 mutations.[15]

DDx:

  • Secretory granules (WC/jensflorian)

  • PAS-positive secretory granules (WC/jensflorian)

  • Smear with secretory granules (WC/jensflorian)

Images:

Lymphoplasmacyte-rich meningioma

Microscopic:

  • Lymphocytes.
  • Plasma cells.

Images:

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

Microscopic:

  • Cartilage or bone formation.

Grade II

Brain invasive meningioma
  • Invades the brain.

Images:

Clear cell meningioma

Epidemiology:

  • Usu. spinal cord.[17]

Microscopic:

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

Images:

Chordoid meningioma
  • Chordoma-like.

Microscopic:

  • Chordoid meningioma - HE (WC/jensflorian)

  • Chordoid meningioma - alcian blue (WC/jensflorian)


Image:

Grade III

Papillary meningioma

Microscopic:

  • discohesive meningothelial tumour cells around a fibrovascular core.
  • perivascular pseudorosettes.

  • Papillary meningioma (WC/jensflorian)

  • Papillary meningioma (WC)

Rhabdoid meningioma

Microscopic:

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

  • Rhabdoid meningioma. (WC/Marvin 101)

  • Frozen section (WC/jensflorian)

www:

Histologic grading

Grading:[13]

  • 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.
      • Invasion 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.[18]
  • Other CKs usually -ve.

DDx of meningioma & IHC[19]

A standard work-up

  • Ki-67 >5-10% - predicts re-occurrence.[21]
  • PR (progesterone receptor) +ve in > 80% of meningiomas.[22]
    • Loss of PR staining predicts recurrence.
    • Strong association with tumour grade:[23]
      • Low WHO grade tumours usu. +ve.
      • High WHO grade tumours usu. -ve.

See also

References

  1. ↑ Rogers, L.; Barani, I.; Chamberlain, M.; Kaley, TJ.; McDermott, M.; Raizer, J.; Schiff, D.; Weber, DC. et al. (Oct 2014). "Meningiomas: knowledge base, treatment outcomes, and uncertainties. A RANO review.". J Neurosurg: 1-20. doi:10.3171/2014.7.JNS131644. PMID 25343186.
  2. ↑ Baldi, I.; Engelhardt, J.; Bonnet, C.; Bauchet, L.; Berteaud, E.; Grüber, A.; Loiseau, H. (Sep 2014). "Epidemiology of meningiomas.". Neurochirurgie. doi:10.1016/j.neuchi.2014.05.006. PMID 25249493.
  3. ↑ URL: http://moon.ouhsc.edu/kfung/jty1/neurotest/Q13-Ans.htm. Accessed on: 26 October 2010.
  4. ↑ Kimonis, VE.; Mehta, SG.; Digiovanna, JJ.; Bale, SJ.; Pastakia, B.. "Radiological features in 82 patients with nevoid basal cell carcinoma (NBCC or Gorlin) syndrome.". Genet Med 6 (6): 495-502. doi:10.109701.GIM.0000145045.17711.1C. PMID 15545745.
  5. ↑ Lee, CW.; Tan, TC. (Feb 2014). "Meningioma associated with Gorlin's syndrome.". J Clin Neurosci 21 (2): 349-50. doi:10.1016/j.jocn.2013.02.033. PMID 24100109.
  6. ↑ URL: http://path.upmc.edu/cases/case702.html. Accessed on: 2 February 2012.
  7. ↑ Ikeda, D.; Chiocca, EA. (Oct 2012). "Editorial: dural tail sign.". J Neurosurg 117 (4): 643-4. doi:10.3171/2012.2.JNS12266. PMID 22839655.
  8. ↑ Wen, M.; Jung, S.; Moon, KS.; Pei, J.; Lee, KH.; Jin, SG.; Li, SY.; Ryu, HH. (Dec 2014). "Immunohistochemical profile of the dural tail in intracranial meningiomas.". Acta Neurochir (Wien) 156 (12): 2263-73. doi:10.1007/s00701-014-2216-4. PMID 25238986.
  9. ↑ Aoki, S.; Sasaki, Y.; Machida, T.; Tanioka, H.. "Contrast-enhanced MR images in patients with meningioma: importance of enhancement of the dura adjacent to the tumor.". AJNR Am J Neuroradiol 11 (5): 935-8. PMID 2120998.
  10. ↑ Qi, ST.; Liu, Y.; Pan, J.; Chotai, S.; Fang, LX. (Oct 2012). "A radiopathological classification of dural tail sign of meningiomas.". J Neurosurg 117 (4): 645-53. doi:10.3171/2012.6.JNS111987. PMID 22839654.
  11. ↑ Goyal, N.; Kakkar, A.; Sarkar, C.; Agrawal, D.. "Does bony hyperostosis in intracranial meningioma signify tumor invasion? A radio-pathologic study.". Neurol India 60 (1): 50-4. doi:10.4103/0028-3886.93589. PMID 22406780.
  12. ↑ URL: http://radiographics.rsna.org/content/23/3/785.long. Accessed on: 3 November 2010.
  13. ↑ 13.0 13.1 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.
  14. ↑ URL: http://moon.ouhsc.edu/kfung/jty1/Com04/Com405-1-Diss.htm. Accessed on: 12 October 2011.
  15. ↑ Reuss, DE.; Piro, RM.; Jones, DT.; Simon, M.; Ketter, R.; Kool, M.; Becker, A.; Sahm, F. et al. (Mar 2013). "Secretory meningiomas are defined by combined KLF4 K409Q and TRAF7 mutations.". Acta Neuropathol 125 (3): 351-8. doi:10.1007/s00401-013-1093-x. PMID 23404370.
  16. ↑ URL: http://moon.ouhsc.edu/kfung/jty1/Com04/Com405-1-Diss.htm. Accessed on: 3 January 2012.
  17. ↑ 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.
  18. ↑ 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.
  19. ↑ 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.
  20. ↑ Grunberg, SM. (1991). "The role of progesterone receptors in meningioma.". Cancer Treat Res 58: 127-37. PMID 1683782.
  21. ↑ Croul, SE. 8 November 2010.
  22. ↑ 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.
  23. ↑ 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.
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