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The role of imaging features and resection status in the survival outcome of sporadic optic pathway glioma children receiving different adjuvant treatments

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Abstract

Optic pathway glioma (OPG) is a rare brain tumor affecting children, with no standard treatment strategy. This study described the sporadic OPG survival outcomes after surgical treatment and analyzed the role of imaging features and resection status in children receiving different adjuvant treatments. This retrospective study included 165 OPG patients whose clinical information were obtained from the hospital record system. Tumor volume and residual tumor volume were calculated by delineating the lesion area. Kaplan–Meier method and Cox proportional hazards model were conducted to analyze the independent prognosis factor. A total of 165 patients were included in this study. Respectively, the 5-year overall survival (OS) and progression-free survival (PFS) were 87.58% and 77.87%. Residual tumor size and first adjuvant treatment (AT) after surgery were both associated with PFS. In patients with small-size residual tumors, there was no significant difference in PFS between the AT treatment groups. Moreover, age, exophytic cystic components, leptomeningeal metastases, and AT were associated with OS. In patients with exophytic cystic components and those with leptomeningeal metastases, there was no significant difference in OS. Our results revealed that OPG patients could avoid or defer AT by maximized resection. Age ≤ 2 years was a disadvantageous factor for OS. Patients with exophytic cystic components were more likely to benefit from primary surgery, and CT or RT was not beneficial for these patients. Patients with leptomeningeal metastases had a poor prognosis regardless of the treatment they received. Future prospective clinical studies are needed to develop more effective treatment regimens.

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The processed data required to reproduce these findings cannot be shared at this time as they are part of an ongoing study.

Abbreviations

AT:

Adjuvant treatment

BMI:

Body mass index

CI:

Confidence interval

CT:

Chemotherapy

DA:

Diffuse astrocytoma

GTR:

Gross total resection

HR:

Hazard ratio

ICH:

Intracranial hypertension

LGG:

Low-grade glioma

LP:

Light perception

NLP:

No light perception

OB:

Observation

OPG:

Optic pathway glioma

OS:

Overall survival

PA:

Pilocytic astrocytoma

PA/PMXA:

Pilocytic astrocytoma with pilomyxoid features

PD:

Progressive disease

PFS:

Progression-free survival

PMXA:

Pilomyxoid astrocytoma

RT:

Radiotherapy

References

  1. Ahn Y, Cho B-K, Kim S-K, Chung Y-N, Lee CS, Kim IH, Yang SW, Kim H-S, Kim HJ, Jung H-W, Wang K-C (2006) Optic pathway glioma: outcome and prognostic factors in a surgical series. Child’s Nerv Syst 22:1136–1142. https://doi.org/10.1007/s00381-006-0086-7

    Article  Google Scholar 

  2. Ater JL, Zhou T, Holmes E, Mazewski CM, Booth TN, Freyer DR, Lazarus KH, Packer RJ, Prados M, Sposto R, Vezina G, Wisoff JH, Pollack IF (2012) Randomized study of two chemotherapy regimens for treatment of low-grade glioma in young children: a report from the Children’s Oncology Group. J Clin Oncol 30:2641–2647. https://doi.org/10.1200/JCO.2011.36.6054

    Article  PubMed  PubMed Central  Google Scholar 

  3. Azizi AA, Schouten-van Meeteren AYN (2018) Current and emerging treatment strategies for children with progressive chiasmatic-hypothalamic glioma diagnosed as infants: a web-based survey. J Neurooncol 136:127–134. https://doi.org/10.1007/s11060-017-2630-6

    Article  CAS  PubMed  Google Scholar 

  4. Barker FG, Curry WT, Carter BS (2005) Surgery for primary supratentorial brain tumors in the United States, 1988 to 2000: the effect of provider caseload and centralization of care. Neuro Oncol 7:49–63. https://doi.org/10.1215/S1152851704000146

    Article  PubMed  PubMed Central  Google Scholar 

  5. Combs SE, Schulz-Ertner D, Moschos D, Thilmann C, Huber PE, Debus J (2005) Fractionated stereotactic radiotherapy of optic pathway gliomas: tolerance and long-term outcome. Int J Radiat Oncol Biol Phys 62:814–819. https://doi.org/10.1016/j.ijrobp.2004.12.081

    Article  PubMed  Google Scholar 

  6. Elsamadicy AA, Sergesketter A, Sampson JH, Gottfried ON (2018) Institutional review of mortality in 5434 consecutive neurosurgery patients: are we improving? Neurosurgery 83:1269–1276. https://doi.org/10.1093/neuros/nyx603

    Article  PubMed  Google Scholar 

  7. Erkal HŞ, Serin M, Çakmak A (1997) Management of optic pathway and chiasmatic-hypothalamic gliomas in children with radiation therapy. Radiother Oncol 45:11–15. https://doi.org/10.1016/S0167-8140(97)00102-3

    Article  CAS  PubMed  Google Scholar 

  8. Fried I, Tabori U, Tihan T, Reginald A, Bouffet E (2013) Optic pathway gliomas: a review. CNS Oncol 2:143–159. https://doi.org/10.2217/cns.12.47

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Gnekow AK, Falkenstein F, von Hornstein S, Zwiener I, Berkefeld S, Bison B, Warmuth-Metz M, Driever PH, Soerensen N, Kortmann R-D, Pietsch T, Faldum A (2012) Long-term follow-up of the multicenter, multidisciplinary treatment study HIT-LGG-1996 for low-grade glioma in children and adolescents of the German Speaking Society of Pediatric Oncology and Hematology. Neuro Oncol 14:1265–1284. https://doi.org/10.1093/neuonc/nos202

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  10. Gnekow AK, Kortmann R-D, Pietsch T, Emser A (2004) Low grade chiasmatic-hypothalamic glioma - carboplatin and vincristin chemotherapy effectively defers radiotherapy within a comprehensive treatment strategy: report from the multicenter treatment study for children and adolescents with a low grade glioma - HIT-LGG 1996 - of the Society of Pediatric Oncology and Hematology (GPOH). Klin Padiatr 216:331–342. https://doi.org/10.1055/s-2004-832355

    Article  CAS  PubMed  Google Scholar 

  11. Gnekow AK, Walker DA, Kandels D, Picton S, Perilongo G, Grill J, Stokland T, Sandstrom PE, Warmuth-Metz M, Pietsch T, Giangaspero F, Schmidt R, Faldum A, Kilmartin D, De Paoli A, De Salvo GL, Gnekow AK, Slavc I, Perilongo G, Picton S, Walker D, Stokland T, Sandstrom PE, Clausen N, Arola M, Jonsson OG, Cruz O, Navajas A, Teijeiro A, Grill J, Kalifa C, Raquin M-A, Verlooy J, Hans V, Pietsch T, Scheurlen W, Hainfellner J, Giangaspero F, Ironside J, Robson K, Skullerud K, Scheie D, Nn R-M, Jouvet A, Figarella-Branger D, Lellouch-Toubiana A, Warmuth-Metz M, Prayer D, Calderone M, Jaspan T, Bakke SJ, Vazquez E, Couanet D, Kortmann RD, Diekmann K, Scarzello G, Taylor R, Lote K, Giralt J, Carrie C, Habrand JL, Soerensen N, Czech T, Chumas P, Gustavson B, Zerah M, Wabbels B, Pinello ML, Fielder A, Simmons I, Christoffersen T, Calaminus G, Brockmann K, Straeter R, Ebinger F, Hernaiz-Driever P, Lackner H, Kennedy C, Glaser A, Stromberg B, Indiano JM, Rodary C, Bouffet E, Frappaz D, Faldum A, Emser A, De Salvo GL, Stephens S, Machin D, Le Deley M-C, Egeland T, Freemann C, Schrappe M, Sposto R (2017) A European randomised controlled trial of the addition of etoposide to standard vincristine and carboplatin induction as part of an 18-month treatment programme for childhood (≤16 years) low grade glioma – a final report. Eur J Cancer 81:206–225. https://doi.org/10.1016/j.ejca.2017.04.019

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  12. Hidalgo ET, Kvint S, Orillac C, North E, Dastagirzada Y, Chang JC, Addae G, Jennings TS, Snuderl M, Wisoff JH (2019) Long-term clinical and visual outcomes after surgical resection of pediatric pilocytic/pilomyxoid optic pathway gliomas. J Neurosurg Pediatr 24:166–173. https://doi.org/10.3171/2019.2.PEDS18529

    Article  PubMed  Google Scholar 

  13. Hollon T, Hervey-Jumper SL, Sagher O, Orringer DA (2015) Advances in the surgical management of low-grade glioma. Semin Radiat Oncol 25:181–188. https://doi.org/10.1016/j.semradonc.2015.02.007

    Article  PubMed  PubMed Central  Google Scholar 

  14. Jenkin D, Angyalfi S, Becker L, Berry M, Buncic R, Chan H, Doherty M, Drake J, Greenberg M, Hendrick B, Hoffman H, Humphreys R, Weitzman S (1993) Optic glioma in children: surveillance, resection, or irradiation? Int J Radiat Oncol Biol Phys 25:215–225. https://doi.org/10.1016/0360-3016(93)90342-S

    Article  CAS  PubMed  Google Scholar 

  15. Konovalov A, Gorelyshev S, Serova N (1994) Surgery of giant gliomas of chiasma and IIIrd ventricle. Acta Neurochir 130:71–79. https://doi.org/10.1007/BF01405505

    Article  CAS  PubMed  Google Scholar 

  16. Laithier V, Grill J, Le Deley M-C, Ruchoux M-M, Couanet D, Doz F, Pichon F, Rubie H, Frappaz D, Vannier J-P, Babin-Boilletot A, Sariban E, Chastagner P, Zerah M, Raquin M-A, Hartmann O, Kalifa C (2003) Progression-free survival in children with optic pathway tumors: dependence on age and the quality of the response to chemotherapy—results of the first French prospective study for the French Society of Pediatric Oncology. J Clin Oncol 21:4572–4578. https://doi.org/10.1200/JCO.2003.03.043

    Article  CAS  PubMed  Google Scholar 

  17. Liao C, Zhang H, Liu Z, Han Z, Li C, Gong J, Liu W, Ma Z, Tian Y (2020) The visual acuity outcome and relevant factors affecting visual improvement in pediatric sporadic chiasmatic–hypothalamic glioma patients who received surgery. Front Neurol 11.https://doi.org/10.3389/fneur.2020.00766

  18. Liu Y, Hao X, Liu W, Li C, Gong J, Ma Z, Tian Y (2018) Analysis of survival prognosis for children with symptomatic optic pathway gliomas who received surgery. World Neurosurg 109:e1–e15. https://doi.org/10.1016/j.wneu.2017.09.144

    Article  PubMed  Google Scholar 

  19. Massimi L, Tufo T, Di Rocco C (2007) Management of optic–hypothalamic gliomas in children: still a challenging problem. Expert Rev Anticancer Ther 7:1591–1610. https://doi.org/10.1586/14737140.7.11.1591

    Article  PubMed  Google Scholar 

  20. Packer RJ, Lange B, Ater J, Nicholson HS, Allen J, Walker R, Prados M, Jakacki R, Reaman G, Needles MN (1993) Carboplatin and vincristine for recurrent and newly diagnosed low-grade gliomas of childhood. J Clin Oncol 11:850–856. https://doi.org/10.1200/JCO.1993.11.5.850

    Article  CAS  PubMed  Google Scholar 

  21. Sawamura Y, Kamada K, Kamoshima Y, Yamaguchi S, Tajima T, Tsubaki J, Fujimaki T (2008) Role of surgery for optic pathway/hypothalamic astrocytomas in children. Neuro Oncol 10:725–733. https://doi.org/10.1215/15228517-2008-033

    Article  PubMed  PubMed Central  Google Scholar 

  22. Scarpa A (1816) Trattato delle principali malattie degli occhi, V. Pavia, nella stamperia di Pietro Bizzoni

  23. Scherer M, Ahmeti H, Roder C, Gessler F, Jungk C, Pala A, Mayer B, Senft C, Tatagiba M, Synowitz M, Wirtz CR, Unterberg AW, Coburger J (2020) Surgery for diffuse WHO grade II gliomas: volumetric analysis of a multicenter retrospective cohort from the German Study Group for intraoperative magnetic resonance imaging. Neurosurgery 86:E64–E74. https://doi.org/10.1093/neuros/nyz397

    Article  PubMed  Google Scholar 

  24. Shofty B, Mauda-Havakuk M, Weizman L, Constantini S, Ben-Bashat D, Dvir R, Pratt L-T, Joskowicz L, Kesler A, Yalon M, Ravid L, Ben-Sira L (2015) The effect of chemotherapy on optic pathway gliomas and their sub-components: a volumetric MR analysis study: volumetric analysis of treated OPG. Pediatr Blood Cancer 62:1353–1359. https://doi.org/10.1002/pbc.25480

    Article  CAS  PubMed  Google Scholar 

  25. Smith JS, Chang EF, Lamborn KR, Chang SM, Prados MD, Cha S, Tihan T, VandenBerg S, McDermott MW, Berger MS (2008) Role of extent of resection in the long-term outcome of low-grade hemispheric gliomas. JCO 26:1338–1345. https://doi.org/10.1200/JCO.2007.13.9337

    Article  Google Scholar 

  26. Steinbok P, Hentschel S, Almqvist P, Cochrane DD, Poskitt K (2002) Management of Optic Chiasmatic/ Hypothalamic Astrocytomas in Children. Can J Neurol Sci 29:132–138. https://doi.org/10.1017/S031716710012089X

    Article  PubMed  Google Scholar 

  27. Stella I, Helleringer M, Joud A, Chastagner P, Thomas R, Klein O (2020) Optic pathway tumor in children: toward a new classification for neurosurgical use. Neurochirurgie S0028377020304665.https://doi.org/10.1016/j.neuchi.2020.11.002

  28. Taylor T, Jaspan T, Milano G, Gregson R, Parker T, Ritzmann T, Benson C, Walker D (2008) Radiological classification of optic pathway gliomas: experience of a modified functional classification system. Br J Radiol 81:761–766. https://doi.org/10.1259/bjr/65246351

    Article  CAS  PubMed  Google Scholar 

  29. Wisoff JH, Abbott R, Epstein F (1990) Surgical management of exophytic chiasmatic-hypothalamic tumors of childhood. J Neurosurg 73:661–667. https://doi.org/10.3171/jns.1990.73.5.0661

    Article  CAS  PubMed  Google Scholar 

  30. Wisoff JH, Sanford RA, Heier LA, Sposto R, Burger PC, Yates AJ, Holmes EJ, Kun LE (2011) Primary neurosurgery for pediatric low-grade gliomas: a prospective multi-institutional study from the Children’s Oncology Group. Neurosurgery 68(6):1548–1554; discussion 1554-5. https://doi.org/10.1227/NEU.0b013e318214a66e

  31. Yeh S-A, Ho J-T, Lui C-C, Huang Y-J, Hsiung C-Y, Huang E-Y (2005) Treatment outcomes and prognostic factors in patients with supratentorial low-grade gliomas. BJR 78:230–235. https://doi.org/10.1259/bjr/28534346

    Article  PubMed  Google Scholar 

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Acknowledgements

We thank all the patients and their families for their cooperation during the follow-up.

Funding

Capital’s Funds for Health Improvement and Research (2020–2-2047).

Author information

Authors and Affiliations

Authors

Contributions

Acquisition of data: ZL, CL, XA, WZ. Analysis and interpretation of data: ZL, CL, XA, WZ. Statistical analysis: ZL, CL. Drafting the article: ZL, CL. Critically revising the article: ZL, C, XA, WZ, ZM, WL, YT. Funding acquisition: YT. Conception and design: ZL, CL, ZM, WL, YT. Study supervision: ZL, CL, ZM, WL, YT.

Corresponding author

Correspondence to Yong-ji Tian.

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Ethics approval

All procedures performed in studies involving human participants with the ethical standards of the institutional and/or national research committee by the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. This study was reviewed and approved by the Institutional Review Board of Beijing Tiantan Hospital, Capital Medical University (KY 2020–054-04).

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Legal guardians of all study participants provided informed written consent to participate.

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All individual case information was removed. The data was analyzed retrospectively after obtaining informed consent from legal guardians of all study participants.

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The authors declare no competing interests.

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Supplementary information

Below is the link to the electronic supplementary material.

Supplementary Fig. S1.

A treatment course of included patients with sporadic optic pathway glioma. (PNG 786 kb)

High resolution image (TIF 4448 kb)

Supplementary Fig. S2.

The progression-free survival (PFS) and overall survival (OS) of all participants. (PNG 81 kb)

High resolution image (TIF 10271 kb)

Supplementary Tables S1–S5

(DOCX 31 kb)

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Liu, Zm., Liao, Ch., An, X. et al. The role of imaging features and resection status in the survival outcome of sporadic optic pathway glioma children receiving different adjuvant treatments. Neurosurg Rev 45, 2277–2287 (2022). https://doi.org/10.1007/s10143-022-01743-1

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