Glioblastoma

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Glioblastoma
Diagnosis in short

Glioblastoma. H&E stain.

LM astrocytic differentiation, nuclear atypia, necrosis, microvascular proliferation, +/-pseudopalisading necrosis
Subtypes gliosarcoma
LM DDx anaplastic astrocytoma
IHC GFAP +ve, IDH-1 -ve/+ve
Site brain, spinal cord

Radiology intra-axial
Prognosis very poor
Clin. DDx metastatic brain tumours

Glioblastoma a very common malignant primary brain tumour in adults. It has a very poor prognosis.

It was previously known as glioblastoma multiforme, abbreviated GBM.

General

  • Median survival is measured in months.[1]
  • Only about 5% can expect to survive more than three years.[2]

Macroscopy

Features:

  • Usu. in white matter.
    • central necrotic core.
    • ill-defined borders.
    • yellowish to dark-brown changes.
    • midline shift due to tumor mass.
  • In the corpus callosum as bihemispheric "butterfly glioma"

Microscopic

Features:

  • Astrocytic tumour with:
    • Nuclear atypia.
    • Necrosis.
    • Endothelial proliferation (AKA microvascular proliferation).
    • +/-"Pseudopalisading necrosis" - tumour cells lined-up like a picket fence around necrotic areas.

Glioblastoma variants:

  • Giant cell glioblastoma (ICD-O: 9441/3)
  • Gliosarcoma (ICD-O: 9442/3)
  • Future variant (in 2016?)
    • Epitheloid glioblastoma (50% BRAF V600E mutations) [3]


Morphological patterns in Glioblastoma:

  • Lipidized (foamy)cells [4]
  • Adipocyte-like maturation [5]
  • Rhabdoid glioblastoma (focal loss of INI-1) [6]
  • Melanotic glioblastoma [7]
  • Glioblastoma with oligodendroglial component (no improved survival) [8]
  • Granular cell Glioblastoma [9]
  • Glioblastoma with PNET component [10]
  • Small cell glioblastoma.
  • Ependymal-like growth patterns.



Images

www:

IHC

  • GFAP +ve (cytoplasm).
  • IDH-1 -ve.
    • +ve if developed from lower grade astrocytoma. (???)

See also

References

  1. Jubelirer, SJ.. "A review of the treatment and survival rates of 138 patients with glioblastoma multiforme.". W V Med J 92 (4): 186-90. PMID 8772403.
  2. Krex, D.; Klink, B.; Hartmann, C.; von Deimling, A.; Pietsch, T.; Simon, M.; Sabel, M.; Steinbach, JP. et al. (Oct 2007). "Long-term survival with glioblastoma multiforme.". Brain 130 (Pt 10): 2596-606. doi:10.1093/brain/awm204. PMID 17785346.
  3. te journal | last1 = Kleinschmidt-DeMasters | first1 = BK. | last2 = Aisner | first2 = DL. | last3 = Birks | first3 = DK. | last4 = Foreman | first4 = NK. | title = Epithelioid GBMs show a high percentage of BRAF V600E mutation. | journal = Am J Surg Pathol | volume = 37 | issue = 5 | pages = 685-98 | month = May | year = 2013 | doi = 10.1097/PAS.0b013e31827f9c5e | PMID = 23552385 }}
  4. Kepes, JJ.; Rubinstein, LJ. (May 1981). "Malignant gliomas with heavily lipidized (foamy) tumor cells: a report of three cases with immunoperoxidase study.". Cancer 47 (10): 2451-9. PMID 7023643.
  5. Rickert, CH.; Riemenschneider, MJ.; Schachenmayr, W.; Richter, HP.; Bockhorn, J.; Reifenberger, G.; Paulus, W. (Jul 2009). "Glioblastoma with adipocyte-like tumor cell differentiation--histological and molecular features of a rare differentiation pattern.". Brain Pathol 19 (3): 431-8. doi:10.1111/j.1750-3639.2008.00199.x. PMID 18691268.
  6. Hiroyuki, M.; Ogino, J.; Takahashi, A.; Hasegawa, T.; Wakabayashi, T. (Feb 2015). "Rhabdoid glioblastoma: an aggressive variaty of astrocytic tumor.". Nagoya J Med Sci 77 (1-2): 321-8. PMID 25797998.
  7. Jaiswal, S.; Agrawal, V.; Vij, M.; Sahu, RN.; Jaiswal, AK.; Behari, S.. "Glioblastoma with melanotic differentiation.". Clin Neuropathol 29 (5): 330-3. PMID 20860897.
  8. Hegi, ME.; Janzer, RC.; Lambiv, WL.; Gorlia, T.; Kouwenhoven, MC.; Hartmann, C.; von Deimling, A.; Martinet, D. et al. (Jun 2012). "Presence of an oligodendroglioma-like component in newly diagnosed glioblastoma identifies a pathogenetically heterogeneous subgroup and lacks prognostic value: central pathology review of the EORTC_26981/NCIC_CE.3 trial.". Acta Neuropathol 123 (6): 841-52. doi:10.1007/s00401-011-0938-4. PMID 22249618.
  9. Schittenhelm, J.; Psaras, T.. "Glioblastoma with granular cell astrocytoma features: a case report and literature review.". Clin Neuropathol 29 (5): 323-9. PMID 20860896.
  10. Perry, A.; Miller, CR.; Gujrati, M.; Scheithauer, BW.; Zambrano, SC.; Jost, SC.; Raghavan, R.; Qian, J. et al. (Jan 2009). "Malignant gliomas with primitive neuroectodermal tumor-like components: a clinicopathologic and genetic study of 53 cases.". Brain Pathol 19 (1): 81-90. doi:10.1111/j.1750-3639.2008.00167.x. PMID 18452568.