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Clinicopathological characteristics and treatment outcomes of epithelioid glioblastoma

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Abstract

Epithelioid glioblastoma is a new variant of glioblastoma that has been recently recognized in the 2016 WHO classification of brain tumors. Given the rarity of epithelioid glioblastoma, the clinical characteristics, pathological features, radiological findings, and treatment outcomes are still not well characterized. Therefore, we identified eighty-four epithelioid glioblastoma cases to investigate these characteristics and identify the possible prognostic factors of survival. There were 55 male and 29 female patients with a mean age of 33.6 years. Headache (77.3%) was the most common clinical symptom, and other common symptoms included nausea or vomiting (34%), dizziness (20.5%), seizures (13.6%), and limb weakness (13.6%). Most lesions (88.1%) were located in cerebral lobes, especially in the frontal lobe and temporal lobe. One hundred percent of the patients were IDH1 wild-type (75/75) and INI-1 positive (58/58), and 57.3% (47/82) of patients harbored BRAFV600E mutation. The median overall survival (OS) of all patients was 10.5 months. Patients who received chemotherapy (p = 0.006) or radiotherapy (p = 0.022) had a longer survival than patients who did not. In addition, the K-M curve showed that the BRAFV600E mutation status was not associated with survival (p = 0.724). These findings may assist clinicians with better understanding and management of epithelioid glioblastoma.

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References

  1. Alexandrescu S, Korshunov A, Lai SH, Dabiri S, Patil S, Li R, Shih CS, Bonnin JM, Baker JA, Du E, Scharnhorst DW, Samuel D, Ellison DW, Perry A (2016) Epithelioid glioblastomas and anaplastic epithelioid pleomorphic xanthoastrocytomas--same entity or first cousins? Brain Pathol 26:215–223. https://doi.org/10.1111/bpa.12295

    Article  PubMed  CAS  Google Scholar 

  2. Babu R, Hatef J, McLendon RE, Cummings TJ, Sampson JH, Friedman AH, Adamson C (2013) Clinicopathological characteristics and treatment of rhabdoid glioblastoma. J Neurosurg 119:412–419. https://doi.org/10.3171/2013.3.JNS121773

    Article  PubMed  Google Scholar 

  3. Behling F, Barrantes-Freer A, Skardelly M, Nieser M, Christians A, Stockhammer F, Rohde V, Tatagiba M, Hartmann C, Stadelmann C, Schittenhelm J (2016) Frequency of BRAF V600E mutations in 969 central nervous system neoplasms. Diagn Pathol 11:55. https://doi.org/10.1186/s13000-016-0506-2

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  4. Birzu C, Tran S, Bielle F, Touat M, Mokhtari K, Younan N, Psimaras D, Hoang-Xuan K, Sanson M, Delattre JY, Idbaih A (2020) Leptomeningeal spread in glioblastoma: diagnostic and therapeutic challenges. Oncologist. 25:e1763–e1776. https://doi.org/10.1634/theoncologist.2020-0258

    Article  PubMed  PubMed Central  Google Scholar 

  5. Broniscer A, Tatevossian RG, Sabin ND, Klimo P Jr, Dalton J, Lee R, Gajjar A, Ellison DW (2014) Clinical, radiological, histological and molecular characteristics of paediatric epithelioid glioblastoma. Neuropathol Appl Neurobiol 40:327–336. https://doi.org/10.1111/nan.12093

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  6. Brown TJ, Brennan MC, Li M, Church EW, Brandmeir NJ, Rakszawski KL, Patel AS, Rizk EB, Suki D, Sawaya R, Glantz M (2016) Association of the extent of resection with survival in glioblastoma: a systematic review and meta-analysis. JAMA Oncol 2:1460–1469. https://doi.org/10.1001/jamaoncol.2016.1373

    Article  PubMed  PubMed Central  Google Scholar 

  7. Brustugun OT, Khattak AM, Tromborg AK, Beigi M, Beiske K, Lund-Iversen M, Helland A (2014) BRAF-mutations in non-small cell lung cancer. Lung Cancer 84:36–38. https://doi.org/10.1016/j.lungcan.2014.01.023

    Article  PubMed  Google Scholar 

  8. Clark KH, Villano JL, Nikiforova MN, Hamilton RL, Horbinski C (2013) 1p/19q testing has no significance in the workup of glioblastomas. Neuropathol Appl Neurobiol 39:706–717. https://doi.org/10.1111/nan.12031

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  9. Davies H, Bignell GR, Cox C, Stephens P, Edkins S, Clegg S, Teague J, Woffendin H, Garnett MJ, Bottomley W, Davis N, Dicks N, Ewing R, Floyd Y, Gray K, Hall S, Hawes R, Hughes J, Kosmidou V, Menzies A, Mould C, Parker A, Stevens C, Watt S, Hooper S, Wilson R, Jayatilake H, Gusterson BA, Cooper C, Shipley J, Hargrave D, Pritchard-Jones K, Maitland N, Chenevix-Trench G, Riggins GJ, Bigner DD, Palmieri G, Cossu A, Flanagan A, Nicholson A, Ho JWC, Leung SY, Yuen ST, Weber BL, Siegler HF, Darrow TL, Paterson H, Marais R, Marshall CJ, Wooster R, Stratton MR, Futreal PA (2002) Mutations of the BRAF gene in human cancer. Nature 417:949–954. https://doi.org/10.1038/Nature00766

    Article  PubMed  CAS  Google Scholar 

  10. Day F, Muranyi A, Singh S, Shanmugam K, Williams D, Byrne D, Pham K, Palmieri M, Tie J, Grogan T, Gibbs P, Sieber O, Waring P, Desai J (2015) A mutant BRAF V600E-specific immunohistochemical assay: correlation with molecular mutation status and clinical outcome in colorectal cancer. Target Oncol 10:99–109. https://doi.org/10.1007/s11523-014-0319-8

    Article  PubMed  Google Scholar 

  11. Elisei R, Viola D, Torregrossa L, Giannini R, Romei C, Ugolini C, Molinaro E, Agate L, Biagini A, Lupi C, Valerio L, Materazzi G, Miccoli P, Piaggi P, Pinchera A, Vitti P, Basolo F (2012) The BRAF(V600E) mutation is an independent, poor prognostic factor for the outcome of patients with low-risk intrathyroid papillary thyroid carcinoma: single-institution results from a large cohort study. J Clin Endocrinol Metab 97:4390–4398. https://doi.org/10.1210/jc.2012-1775

    Article  PubMed  CAS  Google Scholar 

  12. Finneran MM, Georges J, Kakareka M, Moncman R, Enriquez M, Siegal T, Kubicek G, Turtz A, Yocom S, Goldman HW, Barrese J (2019) Epithelioid glioblastoma presenting as aphasia in a young adult with ovarian cancer: a case report. Clin Case Rep 7:821–825. https://doi.org/10.1002/ccr3.2088

    Article  PubMed  PubMed Central  Google Scholar 

  13. Funata N, Nobusawa S, Yamada R, Shinoura N (2016) A case of osteoclast-like giant cell-rich epithelioid glioblastoma with BRAF V600E mutation. Brain Tumor Pathol 33:57–62. https://doi.org/10.1007/s10014-015-0239-z

    Article  PubMed  CAS  Google Scholar 

  14. Furuta T, Miyoshi H, Komaki S, Arakawa F, Morioka M, Ohshima K, Nakada M, Sugita Y (2018) Clinicopathological and genetic association between epithelioid glioblastoma and pleomorphic xanthoastrocytoma. Neuropathology 38:218–227. https://doi.org/10.1111/neup.12459

    Article  PubMed  CAS  Google Scholar 

  15. Gasco J, Franklin B, Fuller GN, Salinas P, Prabhu S (2009) Multifocal epithelioid glioblastoma mimicking cerebral metastasis: case report. Neurocirugia 20:550–554. https://doi.org/10.1016/s1130-1473(09)70133-2

    Article  PubMed  CAS  Google Scholar 

  16. Hegi ME, Diserens AC, Gorlia T, Hamou MF, de Tribolet N, Weller M, Kros JM, Hainfellner JA, Mason W, Mariani L, Bromberg JE, Hau P, Mirimanoff RO, Cairncross JG, Janzer RC, Stupp R (2005) MGMT gene silencing and benefit from temozolomide in glioblastoma. N Engl J Med 352:997–1003. https://doi.org/10.1056/NEJMoa043331

    Article  PubMed  CAS  Google Scholar 

  17. Hervey-Jumper SL, Berger MS (2016) Maximizing safe resection of low- and high-grade glioma. J Neuro-Oncol 130:269–282. https://doi.org/10.1007/s11060-016-2110-4

    Article  Google Scholar 

  18. Huang QL, Cao X, Chai X, Wang X, Xiao C, Wang J (2019) The radiological imaging features of easily misdiagnosed epithelioid glioblastoma in seven patients. World Neurosurg 124:e527–e532. https://doi.org/10.1016/j.wneu.2018.12.128

    Article  Google Scholar 

  19. Ishikawa R, Kadota K, Hayashi T, Kimura N, Inoue K, Ibuki E, Kagawa S, Kushida Y, Okada M, Miyake K, Tamiya T, Nobusawa S, Hirato J, Haba R (2020) A rare case of BRAF V600E-mutated epithelioid glioblastoma with a sarcomatous component. Pathol Int 70:166–170. https://doi.org/10.1111/pin.12896

    Article  PubMed  CAS  Google Scholar 

  20. Kanemaru Y, Natsumeda M, Okada M, Saito R, Kobayashi D, Eda T, Watanabe J, Saito S, Tsukamoto Y, Oishi M, Saito H, Nagahashi M, Sasaki T, Hashizume R, Aoyama H, Wakai T, Kakita A, Fujii Y (2019) Dramatic response of BRAF V600E-mutant epithelioid glioblastoma to combination therapy with BRAF and MEK inhibitor: establishment and xenograft of a cell line to predict clinical efficacy. Acta Neuropathol Commun 7:119. https://doi.org/10.1186/s40478-019-0774-7

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  21. Khanna G, Pathak P, Suri V, Sharma MC, Chaturvedi S, Ahuja A, Bhardwaj M, Garg A, Sarkar C, Sharma R (2018) Immunohistochemical and molecular genetic study on epithelioid glioblastoma: series of seven cases with review of literature. Pathol Res Pract 214:679–685. https://doi.org/10.1016/j.prp.2018.03.019

    Article  PubMed  CAS  Google Scholar 

  22. Kleinschmidt-DeMasters BK, Alassiri AH, Birks DK, Newell KL, Moore W, Lillehei KO (2010) Epithelioid versus rhabdoid glioblastomas are distinguished by monosomy 22 and immunohistochemical expression of INI-1 but not claudin 6. Am J Surg Pathol 34:341–354. https://doi.org/10.1097/PAS.0b013e3181ce107b

    Article  PubMed  Google Scholar 

  23. Kleinschmidt-DeMasters BK, Aisner DL, Birks DK, Foreman NK (2013) Epithelioid GBMs show a high percentage of BRAF V600E mutation. Am J Surg Pathol 37:685–698. https://doi.org/10.1097/PAS.0b013e31827f9c5e

    Article  PubMed  PubMed Central  Google Scholar 

  24. Kohno D, Inoue A, Fukushima M, Aki T, Matsumoto S, Suehiro S, Nishikawa M, Ozaki S, Shigekawa S, Watanabe H, Kitazawa R, Kunieda T (2020) Epithelioid glioblastoma presenting as multicentric glioma: a case report and review of the literature. Surg Neurol Int 11:8. https://doi.org/10.25259/SNI_544_2019

    Article  PubMed  PubMed Central  Google Scholar 

  25. Korshunov A, Golanov A, Sycheva R (2002) Immunohistochemical markers for prognosis of cerebral glioblastomas. J Neuro-Oncol 58:217–236. https://doi.org/10.1023/a:1016218117251

    Article  Google Scholar 

  26. Kuroda J, Nobusawa S, Nakamura H, Yokoo H, Ueda R, Makino K, Yano S, Kuratsu J (2016) A case of an epithelioid glioblastoma with the BRAF V600E mutation colocalized with BRAF intact low-grade diffuse astrocytoma. Neuropathology 36:181–186. https://doi.org/10.1111/neup.12258

    Article  PubMed  CAS  Google Scholar 

  27. Lassaletta A, Zapotocky M, Mistry M, Ramaswamy V, Honnorat M, Krishnatry R, Guerreiro Stucklin A, Zhukova N, Arnoldo A, Ryall S, Ling C, McKeown T, Loukides J, Cruz O, de Torres C, Ho CY, Packer RJ, Tatevossian R, Qaddoumi I, Harreld JH, Dalton JD, Mulcahy-Levy J, Foreman N, Karajannis MA, Wang S, Snuderl M, Nageswara Rao A, Giannini C, Kieran M, Ligon KL, Garre ML, Nozza P, Mascelli S, Raso A, Mueller S, Nicolaides T, Silva K, Perbet R, Vasiljevic A, Faure Conter C, Frappaz D, Leary S, Crane C, Chan A, Ng HK, Shi ZF, Mao Y, Finch E, Eisenstat D, Wilson B, Carret AS, Hauser P, Sumerauer D, Krskova L, Larouche V, Fleming A, Zelcer S, Jabado N, Rutka JT, Dirks P, Taylor MD, Chen S, Bartels U, Huang A, Ellison DW, Bouffet E, Hawkins C, Tabori U (2017) Therapeutic and prognostic implications of BRAF V600E in pediatric low-grade gliomas. J Clin Oncol Off J Am Soc Clin Oncol 35:2934–2941. https://doi.org/10.1200/JCO.2016.71.8726

    Article  CAS  Google Scholar 

  28. Le BH, Close RA (2018) Imaging and histopathologic nuances of epithelioid glioblastoma. Case Rep Surg 2018:1285729–1285725. https://doi.org/10.1155/2018/1285729

    Article  PubMed  PubMed Central  Google Scholar 

  29. Leaver KE, Zhang N, Ziskin JL, Vogel H, Recht L, Thomas RP (2016) Response of metastatic glioma to vemurafenib. Neuro-oncol Pract 3:268–271. https://doi.org/10.1093/nop/npv054

    Article  Google Scholar 

  30. Liu C, Chen T, Liu Z (2016) Associations between BRAF(V600E) and prognostic factors and poor outcomes in papillary thyroid carcinoma: a meta-analysis. World J Surg Oncol 14:241. https://doi.org/10.1186/s12957-016-0979-1

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  31. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, Scheithauer BW, Kleihues P (2007) The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol 114:97–109. https://doi.org/10.1007/s00401-007-0243-4

    Article  PubMed  PubMed Central  Google Scholar 

  32. Louis DN, Perry A, Reifenberger G, von Deimling A, Figarella-Branger D, Cavenee WK, Ohgaki H, Wiestler OD, Kleihues P, Ellison DW (2016) The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol 131:803–820. https://doi.org/10.1007/s00401-016-1545-1

    Article  PubMed  Google Scholar 

  33. Lu VM, George ND, Brown DA, Akinduro OO, Raghunathan A, Jentoft M, Quinones-Hinojosa A, Chaichana KL (2019) Confirming diagnosis and effective treatment for rare epithelioid glioblastoma variant: an integrated survival analysis of the literature. World Neurosurg 131:243–251 e242. https://doi.org/10.1016/j.wneu.2019.08.007

    Article  PubMed  Google Scholar 

  34. Matsumura N, Nakajima N, Yamazaki T, Nagano T, Kagoshima K, Nobusawa S, Ikota H, Yokoo H (2017) Concurrent TERT promoter and BRAF V600E mutation in epithelioid glioblastoma and concomitant low-grade astrocytoma. Neuropathology 37:58–63. https://doi.org/10.1111/neup.12318

    Article  PubMed  CAS  Google Scholar 

  35. Miyahara M, Nobusawa S, Inoue M, Okamoto K, Mochizuki M, Hara T (2016) Glioblastoma with rhabdoid features: report of two young adult cases and review of the literature. World Neurosurg 86(515):e511–e519. https://doi.org/10.1016/j.wneu.2015.10.065

    Article  Google Scholar 

  36. Nabors LB, Portnow J, Ammirati M, Baehring J, Brem H, Brown P, Butowski N, Chamberlain MC, Fenstermaker RA, Friedman A, Gilbert MR, Hattangadi-Gluth J, Holdhoff M, Junck L, Kaley T, Lawson R, Loeffler JS, Lovely MP, Moots PL, Mrugala MM, Newton HB, Parney I, Raizer JJ, Recht L, Shonka N, Shrieve DC, Sills AK Jr, Swinnen LJ, Tran D, Tran N, Vrionis FD, Weiss S, Wen PY, McMillian N, Engh AM (2015) Central nervous system cancers, version 1.2015. J Natl Compr Cancer Netw 13:1191–1202. https://doi.org/10.6004/jnccn.2015.0148

    Article  CAS  Google Scholar 

  37. Nakagomi N, Sakamoto D, Hirose T, Takagi T, Murase M, Nakagomi T, Yoshimura S, Hirota S (2020) Epithelioid glioblastoma with microglia features: potential for novel therapy. Brain Pathol 30:1119–1133. https://doi.org/10.1111/bpa.12887

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  38. Nakajima N, Nobusawa S, Nakata S, Nakada M, Yamazaki T, Matsumura N, Harada K, Matsuda H, Funata N, Nagai S, Nakamura H, Sasaki A, Akimoto J, Hirato J, Yokoo H (2018) BRAF V600E, TERT promoter mutations and CDKN2A/B homozygous deletions are frequent in epithelioid glioblastomas: a histological and molecular analysis focusing on intratumoral heterogeneity. Brain Pathol 28:663–673. https://doi.org/10.1111/bpa.12572

    Article  PubMed  CAS  Google Scholar 

  39. Nitta N, Moritani S, Fukami T, Yoshimura Y, Hirai H, Nozaki K (2018) Intraventricular epithelioid glioblastoma: a case report. World Neurosurg 112:257–263. https://doi.org/10.1016/j.wneu.2018.01.200

    Article  PubMed  Google Scholar 

  40. Nobusawa S, Hirato J, Kurihara H, Ogawa A, Okura N, Nagaishi M, Ikota H, Yokoo H, Nakazato Y (2014) Intratumoral heterogeneity of genomic imbalance in a case of epithelioid glioblastoma with BRAF V600E mutation. Brain Pathol 24:239–246. https://doi.org/10.1111/bpa.12114

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  41. Oldberg E (1933) Hemorrhage into gliomas - a review of eight hundred and thirty-two consecutive verified cases of glioma. Arch Neurol Psychiatr 30:1061–1073. https://doi.org/10.1001/archneurpsyc.1933.02240170113007

    Article  Google Scholar 

  42. Olson RA, Brastianos PK, Palma DA (2011) Prognostic and predictive value of epigenetic silencing of MGMT in patients with high grade gliomas: a systematic review and meta-analysis. J Neuro-Oncol 105:325–335. https://doi.org/10.1007/s11060-011-0594-5

    Article  Google Scholar 

  43. Ostrom QT, Gittleman H, Truitt G, Boscia A, Kruchko C, Barnholtz-Sloan JS (2018) CBTRUS Statistical Report: primary brain and other central nervous system tumors diagnosed in the United States in 2011-2015. Neuro-oncology 20:iv1–iv86. https://doi.org/10.1093/neuonc/noy131

    Article  PubMed  PubMed Central  Google Scholar 

  44. Pratt D, Pittaluga S, Palisoc M, Fetsch P, Xi L, Raffeld M, Gilbert MR, Quezado M (2017) Expression of CD70 (CD27L) is associated with epithelioid and sarcomatous features in IDH-wild-type glioblastoma. J Neuropathol Exp Neurol 76:697–708. https://doi.org/10.1093/jnen/nlx051

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  45. Rodriguez FJ, Scheithauer BW, Giannini C, Bryant SC, Jenkins RB (2008) Epithelial and pseudoepithelial differentiation in glioblastoma and gliosarcoma: a comparative morphologic and molecular genetic study. Cancer 113:2779–2789. https://doi.org/10.1002/cncr.23899

    Article  PubMed  Google Scholar 

  46. Safaee Ardekani G, Jafarnejad SM, Tan L, Saeedi A, Li G (2012) The prognostic value of BRAF mutation in colorectal cancer and melanoma: a systematic review and meta-analysis. PLoS One 7:e47054. https://doi.org/10.1371/journal.pone.0047054

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  47. Schindler G, Capper D, Meyer J, Janzarik W, Omran H, Herold-Mende C, Schmieder K, Wesseling P, Mawrin C, Hasselblatt M, Louis DN, Korshunov A, Pfister S, Hartmann C, Paulus W, Reifenberger G, von Deimling A (2011) Analysis of BRAF V600E mutation in 1,320 nervous system tumors reveals high mutation frequencies in pleomorphic xanthoastrocytoma, ganglioglioma and extra-cerebellar pilocytic astrocytoma. Acta Neuropathol 121:397–405. https://doi.org/10.1007/s00401-011-0802-6

    Article  PubMed  CAS  Google Scholar 

  48. Schwartzentruber J, Korshunov A, Liu XY, Jones DT, Pfaff E, Jacob K, Sturm D, Fontebasso AM, Quang DA, Tonjes M, Hovestadt V, Albrecht S, Kool M, Nantel A, Konermann C, Lindroth A, Jager N, Rausch T, Ryzhova M, Korbel JO, Hielscher T, Hauser P, Garami M, Klekner A, Bognar L, Ebinger M, Schuhmann MU, Scheurlen W, Pekrun A, Fruhwald MC, Roggendorf W, Kramm C, Durken M, Atkinson J, Lepage P, Montpetit A, Zakrzewska M, Zakrzewski K, Liberski PP, Dong Z, Siegel P, Kulozik AE, Zapatka M, Guha A, Malkin D, Felsberg J, Reifenberger G, von Deimling A, Ichimura K, Collins VP, Witt H, Milde T, Witt O, Zhang C, Castelo-Branco P, Lichter P, Faury D, Tabori U, Plass C, Majewski J, Pfister SM, Jabado N (2012) Driver mutations in histone H3.3 and chromatin remodelling genes in paediatric glioblastoma. Nature 482:226–231. https://doi.org/10.1038/nature10833

    Article  PubMed  CAS  Google Scholar 

  49. Shaw EG, Wisoff JH (2003) Prospective clinical trials of intracranial low-grade glioma in adults and children. Neuro-oncology 5:153–160. https://doi.org/10.1215/S1152851702000601

    Article  PubMed  PubMed Central  Google Scholar 

  50. Silantyev AS, Falzone L, Libra M, Gurina OI, Kardashova KS, Nikolouzakis TK, Nosyrev AE, Sutton CW, Mitsias PD, Tsatsakis A (2019) Current and future trends on diagnosis and prognosis of glioblastoma: from molecular biology to proteomics. Cells 8. https://doi.org/10.3390/cells8080863

  51. Smith-Cohn M, Davidson C, Colman H, Cohen AL (2019) Challenges of targeting BRAF V600E mutations in adult primary brain tumor patients: a report of two cases. CNS Oncol 8:CNS48. https://doi.org/10.2217/cns-2019-0018

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  52. Smits M (2016) Imaging of oligodendroglioma. Br J Radiol 89:20150857. https://doi.org/10.1259/bjr.20150857

    Article  PubMed  PubMed Central  Google Scholar 

  53. Stupp R, Hegi ME, Mason WP, van den Bent MJ, Taphoorn MJ, Janzer RC, Ludwin SK, Allgeier A, Fisher B, Belanger K, Hau P, Brandes AA, Gijtenbeek J, Marosi C, Vecht CJ, Mokhtari K, Wesseling P, Villa S, Eisenhauer E, Gorlia T, Weller M, Lacombe D, Cairncross JG, Mirimanoff RO, European Organisation for R, Treatment of Cancer Brain T, Radiation Oncology G, National Cancer Institute of Canada Clinical Trials G (2009) Effects of radiotherapy with concomitant and adjuvant temozolomide versus radiotherapy alone on survival in glioblastoma in a randomised phase III study: 5-year analysis of the EORTC-NCIC trial. Lancet Oncol 10:459–466. https://doi.org/10.1016/S1470-2045(09)70025-7

    Article  PubMed  CAS  Google Scholar 

  54. Sugimoto K, Ideguchi M, Kimura T, Kajiwara K, Imoto H, Sadahiro H, Ishii A, Kawano H, Ikeda E, Suzuki M (2016) Epithelioid/rhabdoid glioblastoma: a highly aggressive subtype of glioblastoma. Brain Tumor Pathol 33:137–146. https://doi.org/10.1007/s10014-015-0243-3

    Article  PubMed  CAS  Google Scholar 

  55. Tanaka S, Nakada M, Nobusawa S, Suzuki SO, Sabit H, Miyashita K, Hayashi Y (2014) Epithelioid glioblastoma arising from pleomorphic xanthoastrocytoma with the BRAF V600E mutation. Brain Tumor Pathol 31:172–176. https://doi.org/10.1007/s10014-014-0192-2

    Article  PubMed  Google Scholar 

  56. Thakkar JP, Dolecek TA, Horbinski C, Ostrom QT, Lightner DD, Barnholtz-Sloan JS, Villano JL (2014) Epidemiologic and molecular prognostic review of glioblastoma. Cancer Epidemiol Biomark Prev 23:1985–1996. https://doi.org/10.1158/1055-9965.EPI-14-0275

    Article  CAS  Google Scholar 

  57. Tosuner Z, Gecer MO, Hatiboglu MA, Abdallah A, Turna S (2018) BRAF V600E mutation and BRAF VE1 immunoexpression profiles in different types of glioblastoma. Oncol Lett 16:2402–2408. https://doi.org/10.3892/ol.2018.8919

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  58. Wakai S, Yamakawa K, Manaka S, Takakura K (1982) Spontaneous intracranial hemorrhage caused by brain tumor: its incidence and clinical significance. Neurosurgery 10:437–444. https://doi.org/10.1227/00006123-198204000-00004

    Article  PubMed  CAS  Google Scholar 

  59. Wang S, He Q, Zhang Q, Guan B, Zhou X (2020) Clinicopathologic features and prognosis of epithelioid glioblastoma. Int J Clin Exp Pathol 13:1529–1539

    PubMed  PubMed Central  Google Scholar 

  60. Weller M, van den Bent M, Hopkins K, Tonn JC, Stupp R, Falini A, Cohen-Jonathan-Moyal E, Frappaz D, Henriksson R, Balana C, Chinot O, Ram Z, Reifenberger G, Soffietti R, Wick W, European Association for Neuro-Oncology Task Force on Malignant G (2014) EANO guideline for the diagnosis and treatment of anaplastic gliomas and glioblastoma. Lancet Oncol 15:e395–e403. https://doi.org/10.1016/S1470-2045(14)70011-7

    Article  PubMed  Google Scholar 

  61. Werner JM, Brunn A, Fink GR, Galldiks N (2019) Magnetic resonance imaging reveals a pronounced treatment response of a isocitrate dehydrogenase- and B-Raf proto-oncogene-wildtype epithelioid glioblastoma. World Neurosurg 127:213–215. https://doi.org/10.1016/j.wneu.2019.04.025

    Article  PubMed  Google Scholar 

  62. Woo PYM, Lam TC, Pu JKS, Li LF, Leung RCY, Ho JMK, Zhung JTF, Wong B, Chan TSK, Loong HHF, Ng HK (2019) Regression of BRAF (V600E) mutant adult glioblastoma after primary combined BRAF-MEK inhibitor targeted therapy: a report of two cases. Oncotarget 10:3818–3826

    Article  Google Scholar 

  63. Zeng Y, Zhu X, Wang Y, Liu B, Yang X, Wang Q, Du J, Ma Y, Lin L, Fu P, Xiao H, Guo QN (2020) Clinicopathological, immunohistochemical and molecular genetic study on epithelioid glioblastoma: a series of fifteen cases with literature review. OncoTargets Ther 13:3943–3952. https://doi.org/10.2147/OTT.S249317

    Article  CAS  Google Scholar 

  64. Zhang RQ, Shi Z, Chen H, Chung NY, Yin Z, Li KK, Chan DT, Poon WS, Wu J, Zhou L, Chan AK, Mao Y, Ng HK (2016) Biomarker-based prognostic stratification of young adult glioblastoma. Oncotarget 7:5030–5041. https://doi.org/10.18632/oncotarget.5456

    Article  PubMed  Google Scholar 

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This study was supported by the Key Research and Development Project from the Department of Science and Technology of Sichuan Province, China (No. 2017SZ0006).

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Contributions

Study conception and design: KJS and YHL. Data acquisition: KJS, XWZ, TFL, MRZ, and JHL. Analysis and interpretation of data: KJS and XWZ. Statistical analysis: KJS and XWZ. Manuscript preparation and editing: KJS and YHL.

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Correspondence to Yanhui Liu.

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This study was approved by the Ethics Committee of West China Hospital.

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Informed consent form for all included cases in our institution was obtained.

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All authors approved the manuscript to be published.

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The authors declare no competing interests.

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Sun, K., Zhou, X., Li, T. et al. Clinicopathological characteristics and treatment outcomes of epithelioid glioblastoma. Neurosurg Rev 44, 3335–3348 (2021). https://doi.org/10.1007/s10143-021-01492-7

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  • DOI: https://doi.org/10.1007/s10143-021-01492-7

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