Neuropathology tumours

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A brain stem astrocytoma. (WC)

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.

There are separate articles for peripheral nerve sheath tumours and pituitary/peri-pituitary lesions.

Brain tumours - overview

Alphabetical

For overview see here

By age group

Adult

Four most common types of brain tumours:[1]

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

Children

  1. Pilocytic astrocytoma.
  2. Medulloblastoma.
  3. Ependymoma.

By location

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

Cerebrum

Cerebellum

Sella turcica

less common:

Spinal cord

Filum terminale

Meninges

less common:

  • Melanoma / Melanocytoma.
  • Lymphoproliferative diseases.
  • Sarcoidosis
  • Arachnoid cyst.
  • Disseminated oligodendroglial-like leptomeningeal tumour.
  • Desmoplastic infantile astrocytoma / ganglioglioma.
  • Meningioangiomatosis.
  • Calcifying pseudoneoplasm.

Skull

Skull base / Cerebellopontine angle

less common:

Cystic tumours

DDx:[3]

Primary versus secondary

  • AKA (primary) brain tumour versus metastatic cancer.

Primary

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.

Lymphoma:

  • Large (lymphoid) cells, ergo usu. not a difficult diagnosis.
    • ~2x size of resting lymphocyte, nucleoli.
  • Lesion predominantly perivascular.

Secondary

Carcinomas:

  • Well-demarcated border between brain and lesion - key feature.
  • No cytoplasmic processes.
  • Usu. have nuclear atypia of malignancy.
    • Nuclei often ~3-4x the size of a RBC.
  • +/-Glandular arrangement.
  • +/-Nucleoli.

Common neuropathology tumours in a table

Type Key feature(s) Imaging History Notes IHC Images
Normal tissue cells regularly spaced, no nuc. atypia small lesion? / deep lesion? variable missed lesion? nil
Normal. (WC)
Reactive astrocytes astrocytes with well-demarcated eosinophilic cytoplasm, regular spacing, no nuc. atypia small lesion? / deep lesion? variable missed lesion / close to a lesion; non-specific pathologic process - need more tissue nil
Reactive astrocytes. (WC)
Schwannoma cellular areas (Antoni A), paucicelluar areas (Antoni B), palisading of nuclei (Verocay bodies) extra-axial + intradural old or young need frozen section to Dx, DDx: meningioma S100
Schwannoma. (WC)
Meningioma whorls, psammomatous calcs, nuclear inclusions extra-axial + intradural old or young may be diagnosed on smear, DDx: schwannoma, choroid plexus EMA, PR, Ki-67
Meningioma. (WC)
Infiltrative astrocytoma (WHO grade II or grade III) glial processes (esp. on smear), nuclear atypia (typical size var. ~3x, irreg. nuc. membrane, hyperchromasia), no Rosenthal fibres in the core of the lesion †, no microvascular proliferation, no necrosis often enhancing (suggests high grade), usu. supratentorial, usu. white matter usu. old, occ. young common IDH-1+/-, GFAP+
High-grade astrocytoma. (WC)
Glioblastoma (WHO grade IV) glial processes (esp. on smear), nuclear atypia (typical size var. ~3x, irreg. nuc. membrane, hyperchromasia), no Rosenthal fibres in the core of the lesion †, microvascular proliferation or necrosis often enhancing (suggests high grade), usu. supratentorial, usu. white matter usu. old, occ. young very common, esp. glioblastoma IDH-1+/-, GFAP+
Glioblastoma. (WC)
Metastasis sharp interface with brain, often glandular, +/-nucleoli, no glial processes often cerebellular, well-circumscribed usu. old often suspected to have metastatic disease TTF-1, CK7, CK20, BRST-2
Metastasis. (WC)

† Rosenthal fibres at the periphery of a lesion are a non-specific finding seen in chronic processes.

Brain metastasis

Main article: Brain metastasis

Infiltrative astrocytomas

Main article: Astrocytoma

Overview

Notes:

Microscopic

Features:[5][6]

  • Glial processes - key feature.
    • Thin stringy cytoplasmic processes - best seen at high power in less cellular areas.
  • No Rosenthal fibres within the tumour itself.

Images:

Notes:

  • Glial vs. non-glial tumours:
    • Glial: "blends into brain"/gradual transition to non-tumour brain.
    • Non-glial: no glial processes.
  • Rosenthal fibres within the tumour... make it into a pilocytic astrocytoma.
    • Rosenthal fibres may be seen around a (very) slow growing tumour and represent a reactive process.
  • Inflammatory cells and macrophages should prompt consideration of an alternate diagnosis (e.g. cerebral infarct, multiple sclerosis) - esp. if this is a primary lesion.[7]

Grading

Nuclear pleomorphism present:

  • At least grade II (diffuse astrocytoma).

Mitotic figures present:

  • At least grade III (anaplastic astrocytoma).

Microvascular proliferation or necrosis with pseudopalisading tumour cells:

  • Grade IV (glioblastoma AKA glioblastoma multiforme).

Notes:

  • Pseudopalisading tumour cells = high tumour cell density adjacent to regions of necrosis; palisade = a fence of poles forming a defensive barrier or fortification.

Images

Glioblastoma:

  • Glioblastoma - pseudopalisading of tumour cells (WC)

  • Glioblastoma with fragment of near-normal white matter - high mag. (WC)

Anaplastic astrocytoma:

  • Anaplastic astrocytoma - very high mag. (WC)

  • Anaplastic astrocytoma - GFAP - very high mag. (WC)

Table of common gliomas - grading

Histomorphologic comparison of common gliomas:

Entity Rosenthal
fibres / EGBs		 Nuclear atypia Mitoses Necrosis or MVP Infiltrative Image
Pilocytic astrocytoma yes usu. no usu. no usu. no no
Rosenthal HE 40x.jpg
Low-grade astrocytoma no yes no no yes
Astrocytoma whoII HE.jpg
Anaplastic astrocytoma no yes yes no yes
Anaplastic astrocytoma - high mag.jpg
Glioblastoma no yes yes yes yes
Glioblastoma - high mag.jpg

Notes:

  • MVP = microvascular proliferation.
  • EGBs = eosinophilic granular bodies.

IHC

  • GFAP - should stain cytoplasm of tumour cells and the perikaryon (nuclear membrane).
  • Ki-67 - usu. high >20% of cells.
  • p53 - often +ve.
  • IDH1 (isocitrate dehydrogenase 1).

Notes:

  • IDH1 and IDH2 mutations - better survival.[9]

Astrocytic tumours

Main article: Astrocytoma

Oligodendroglial tumours

Ependymal tumours

Choroid plexus tumours

Other neuroepithelial tumours

Astroblastoma

  • No WHO grade yet.[10]
  • Very rare superficial tumor of young age.[11]
  • Large, cystic. Pushing margin towards CNS.
  • Vasocentric growth, plump cells with absence of fibrillary pattern.
  • GFAP+ve, Synaptohysin-ve, Olig-2-ve, focally EMA/panCK+ve. MIB-1: 1-18 %.
  • Molecular profile overlaps with classical CNS-PNET.
    • Gene fusions invoving meningioma gene (MN1)[12]


  • HE. (WC/jensflorian)

  • HE. (WC/jensflorian)

  • Astroblastoma (AFIP)

Chordoid glioma of the third ventricle

  • WHO grade II.
  • Slowly growing, non-invasive.
  • Clusters of epithelioid cells in mucinous stroma.
  • Lymphocytic infiltrates, adjacent Rosenthal fibers.
  • Few mitoses.
  • GFAP+ve, MIB-1 1-3%.

Cribiform neuroepithelial tumour

AKA: CRINET.

  • Not listed in the current WHO classification.
  • First description in 2009.[13]
  • Around ventricles.[14]
  • Young children.[15]
  • Small undifferentiated cells arranged in cribriform strands and trabeculae of varying thickness.
  • MAP2+ve, Synaptophysin+ve, CK+/-ve. MIB-1: 30%.
  • INI-1 loss, but no rhabdoid features and good prognosis.
  • Stable genomic profile.[16]

Neuronal and mixed neuronal/glial tumours

Cerebellar liponeurocytoma

  • Previously called lipomatous medulloblastoma (name changed in WHO 2000).
  • Mean age: 50 years.
  • As the name states: A tumour of the cerebellum.
    • But cases outside cerebellum reported that would qualify.[17]
  • WHO grade II [18] (upgraded from WHO grade I in 2007)[19]
  • ICD-O code: 9506/1

Histo

  • Advanced neuronal and lipomatous differentiation.
  • Neurocytes: round to oval nuclei with clear cytoplasm.
  • Quite cellular.
  • Mitoses almost absent.

IHC

  • GFAP +/-ve (focal).
  • MAP2 +ve.
  • Synaptophysin +ve.
  • NeuN +ve.
  • MIB-1: usu 1-3%.

  • Liponeurocytoma, HE (WC/Marvin101).

  • Liponeurocytoma, Synapto (WC/Marvin101).

  • Liponeurocytoma, HE (WC/jensflorian).

  • Liponeurocytoma, Synapto (WC/jensflorian).

DDx

Gangliocytoma

  • Grade I WHO neuronal tumour.
    • ICD-O code: 9492/0
  • Groups of irregular large neurons.
  • Non-neoplastic, reticulin-rich glial stroma.

Ganglioglioma

Not to be confused with ganglioneuroma.

General

  • Grade I WHO mixed neuronal-glial tumour.
  • ICD-O code: 9505/1 (Anaplastic ganglioglioma: 9505/3)
  • Rare.
  • Usu. temporal lobe.
  • Recognized as a cause of epilepsy.[20]

Microscopic

Features:

  • Dysplastic neurons.
    • Out of regular architecture / abnormal location.
    • Cytomegaly
    • Clustering
    • Binucleated (very occassionally).
  • Atypical glia.
  • Calcification.
  • Lymphocytic cuffing.

Anaplastic ganglioglioma:

  • Brisk mitotic activity
  • Necrosis

IHC

  • Neurons:
    • MAP2 +ve
    • Synaptophysin +ve
    • Neurofilament +ve
  • Glia:
    • CD34+/-ve

DDx:

Images

  • Lymphocytic cuffing in ganglioglioma (WC/jensflorian)

  • Calcification in ganglioglioma (WC/jensflorian)

  • CD34 immunostain in ganglioglioma (WC/jensflorian)


Lhermitte-Duclos disease

  • Abbreviated LDD.
  • AKA dysplastic cerebellar gangliocytoma.[21]
  • AKA dysplastic gangliocytoma of the cerebellum.
Main article: Lhermitte-Duclos disease

Papillary glioneuronal tumour

  • Abbreviated PGNT.
  • A benign, supratentorial tumor of childhood.
    • Biologic course corresponds to WHO grade I.
    • Before WHO 2000, considered a Ganglioglioma variant.
  • Prominent pseudopapillary architecture.
  • Neurocytes to medium-sized ganglion cells.
  • GFAP+ core, GFAP- layer
  • Rosenthal fibers, Eosinophilic Granular bodies and lymphocytic cuffing may be present.

  • PGNT (HE) (WC/jensflorian)

Rosette-forming glioneuronal tumour of the fourth ventricle

  • Abbreviated RGNT.
  • Provisional ICD-O code: 9509/1
  • A rare benign infratentorial tumour of the midline of children and adults.
  • Biologic course corresponds to WHO grade I.
  • Glial component corresponds to pilocytic astrocytoma.
  • Neurocytic rosettes.
  • Eosinopil fibrillary cores / pseudorosettes.
  • GFAP+ in fibrillary areas, Syn+ in rosettes.
  • Neurocytic cells: MAP2+
  • MIB-1 usu. below 3%.

  • RGNT, HE stain (WC/jensflorian).

  • RGNT, higher magnification (WC/jensflorian).

Pineal tumours

Main article: Pineal gland

Embryonal tumours

Peripheral nerve sheath tumours

Main article: Peripheral nerve sheath tumours

A classification:[22] Benign:

Malignant:

Ganglioneuroma

Not to be confused with ganglioglioma.
Main article: Ganglioneuroma


Meningioma

Main article: Meningioma

Chordoma

Main article: Chordoma

Hemangioblastoma

Main article: Hemangioblastoma

CNS lymphoma

Classification:

  • Primary CNS lymphoma.
  • Non-primary CNS lymphoma - see lymphoma article.

General - primary CNS

  • Classically periventicular distribution.
  • Usually large B cell; can be considered a type of diffuse large B cell lymphoma (DLBCL).
    • Prognosis of CNS (DLBCL) lymphomas worse than nodal (non-CNS) DLBCL.[24]

Microscopic

Features:

  • Large cell lymphoma.
    • Size = 2x diameter normal lymphocyte.
    • Nucleolus - common.
  • Perivascular clustering.

Images

www:

  • CNS lymphoma - low mag. (WC)

  • CNS lymphoma - intermed. mag. (WC)

  • CNS lymphoma - high mag. (WC)

  • CNS lymphoma - very high mag. (WC)

  • CNS lymphoma. (WC/KGH)

  • CNS lymphoma. (WC/KGH)

IHC

Can be subclassified in GCB (germinal centre B-cell-like) and non-GCB by CD10, Bcl-6, MUM1/IRF-4, and Bcl-2.[24]

Common pattern:

  • CD20 +ve - key stain.
  • CD3 -ve.
  • Ki-67 ~40%.
  • Bcl-6 +ve.
  • Bcl-1 -ve.


Ganglioneuroblastoma

Main article: Neuroblastoma

General

  • Uncommon.
  • Part of the neuroblastic tumours group which includes:[25]

Microscopic

Features:

  • Ganglion-like cells with a prominent nucleolus.
  • Small undifferentiated cells with scant cytoplasm.

  • Adrenal Ganglioneuroblastoma - Low power (SKB)

  • Adrenal Ganglioneuroblastoma - Medium power (SKB)

  • Adrenal Ganglioneuroblastoma - High power (SKB)

  • Adrenal Ganglioneuroblastoma - High power (SKB)

  • Adrenal Ganglioneuroblastoma - High power (SKB)

Images:

IHC

  • NSE +ve -- small cells.

Lesions of the sella turcica

Main article: Pituitary gland

Lesions of the sella turcica, the pituitary gland environs, is a topic for it self. The differential diagnosis for lesions in this area includes:

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. ↑ URL: http://path.upmc.edu/cases/case320/dx.html. Accessed on: 14 January 2012.
  4. ↑ URL: http://www.pathologyoutlines.com/Cnstumor.html#cystsgeneral. Accessed on: 14 January 2012.
  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. ↑ URL: http://path.upmc.edu/cases/case79/dx.html. Accessed on: 2 January 2012.
  8. ↑ Yan H, Parsons DW, Jin G, et al. (February 2009). "IDH1 and IDH2 mutations in gliomas". N. Engl. J. Med. 360 (8): 765–73. doi:10.1056/NEJMoa0808710. PMC 2820383. PMID 19228619. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2820383/.
  9. ↑ Houillier C, Wang X, Kaloshi G, et al. (October 2010). "IDH1 or IDH2 mutations predict longer survival and response to temozolomide in low-grade gliomas". Neurology 75 (17): 1560–6. doi:10.1212/WNL.0b013e3181f96282. PMID 20975057.
  10. ↑ The International Agency for Research on Cancer (Editors: Louis, D.N.; Ohgaki, H.; Wiestler, O.D.; Cavenee, W.K.) (2007). Pathology and Genetics of Tumours of Tumors of the Central Nervous System (IARC WHO Classification of Tumours) (4th ed.). Lyon: World Health Organization. pp. 88. doi:10.1007/s00401-007-0243-4. ISBN 978-9283224303.
  11. ↑ Narayan, S.; Kapoor, A.; Singhal, MK.; Jakhar, SL.; Bagri, PK.; Rajput, PS.; Kumar, HS.. "Astroblastoma of cerebrum: A rare case report and review of literature.". J Cancer Res Ther 11 (3): 667. doi:10.4103/0973-1482.140800. PMID 26458709.
  12. ↑ Sturm, D.; Orr, BA.; Toprak, UH.; Hovestadt, V.; Jones, DT.; Capper, D.; Sill, M.; Buchhalter, I. et al. (Feb 2016). "New Brain Tumor Entities Emerge from Molecular Classification of CNS-PNETs.". Cell 164 (5): 1060-72. doi:10.1016/j.cell.2016.01.015. PMID 26919435.
  13. ↑ Hasselblatt, M.; Oyen, F.; Gesk, S.; Kordes, U.; Wrede, B.; Bergmann, M.; Schmid, H.; Frühwald, MC. et al. (Dec 2009). "Cribriform neuroepithelial tumor (CRINET): a nonrhabdoid ventricular tumor with INI1 loss and relatively favorable prognosis.". J Neuropathol Exp Neurol 68 (12): 1249-55. doi:10.1097/NEN.0b013e3181c06a51. PMID 19915490.
  14. ↑ Arnold, MA.; Stallings-Archer, K.; Marlin, E.; Grondin, R.; Olshefski, R.; Biegel, JA.; Pierson, CR.. "Cribriform neuroepithelial tumor arising in the lateral ventricle.". Pediatr Dev Pathol 16 (4): 301-7. doi:10.2350/12-12-1287-CR.1. PMID 23495723.
  15. ↑ Park, JY.; Kim, E.; Kim, DW.; Chang, HW.; Kim, SP. (Oct 2012). "Cribriform neuroepithelial tumor in the third ventricle: a case report and literature review.". Neuropathology 32 (5): 570-6. doi:10.1111/j.1440-1789.2011.01293.x. PMID 22239490.
  16. ↑ Gessi, M.; Japp, AS.; Dreschmann, V.; Zur Mühlen, A.; Goschzik, T.; Dörner, E.; Pietsch, T. (Oct 2015). "High-Resolution Genomic Analysis of Cribriform Neuroepithelial Tumors of the Central Nervous System.". J Neuropathol Exp Neurol 74 (10): 970-4. doi:10.1097/NEN.0000000000000239. PMID 26352987.
  17. ↑ Gupta, K.; Salunke, P.; Kalra, I.; Vasishta, RK.. "Central liponeurocytoma: case report and review of literature.". Clin Neuropathol 30 (2): 80-5. PMID 21329617.
  18. ↑ Nishimoto, T.; Kaya, B. (Aug 2012). "Cerebellar liponeurocytoma.". Arch Pathol Lab Med 136 (8): 965-9. doi:10.5858/arpa.2011-0337-RS. PMID 22849747.
  19. ↑ Brat, DJ.; Parisi, JE.; Kleinschmidt-DeMasters, BK.; Yachnis, AT.; Montine, TJ.; Boyer, PJ.; Powell, SZ.; Prayson, RA. et al. (Jun 2008). "Surgical neuropathology update: a review of changes introduced by the WHO classification of tumours of the central nervous system, 4th edition.". Arch Pathol Lab Med 132 (6): 993-1007. doi:10.1043/1543-2165(2008)132[993:SNUARO]2.0.CO;2. PMID 18517285.
  20. ↑ Im, SH.; Chung, CK.; Cho, BK.; Lee, SK. (Mar 2002). "Supratentorial ganglioglioma and epilepsy: postoperative seizure outcome.". J Neurooncol 57 (1): 59-66. PMID 12125968.
  21. ↑ Yağci-Küpeli, B.; Oguz, KK.; Bilen, MA.; Yalçin, B.; Akalan, N.; Büyükpamukçu, M. (Mar 2010). "An unusual cause of posterior fossa mass: Lhermitte-Duclos disease.". J Neurol Sci 290 (1-2): 138-41. doi:10.1016/j.jns.2009.12.010. PMID 20060133.
  22. ↑ 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.
  23. ↑ URL: http://medical-dictionary.thefreedictionary.com/ganglioma. Accessed on: 8 November 2010.
  24. ↑ 24.0 24.1 Raoux D, Duband S, Forest F, et al. (June 2010). "Primary central nervous system lymphoma: Immunohistochemical profile and prognostic significance". Neuropathology 30 (3): 232–40. doi:10.1111/j.1440-1789.2009.01074.x. PMID 19925562.
  25. ↑ 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.

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