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Ntenti, C.; Lallas, K.; Papazisis, G. Prognostic Factors of Childhood Medulloblastoma. Encyclopedia. Available online: https://encyclopedia.pub/entry/45635 (accessed on 27 July 2024).
Ntenti C, Lallas K, Papazisis G. Prognostic Factors of Childhood Medulloblastoma. Encyclopedia. Available at: https://encyclopedia.pub/entry/45635. Accessed July 27, 2024.
Ntenti, Charikleia, Konstantinos Lallas, Georgios Papazisis. "Prognostic Factors of Childhood Medulloblastoma" Encyclopedia, https://encyclopedia.pub/entry/45635 (accessed July 27, 2024).
Ntenti, C., Lallas, K., & Papazisis, G. (2023, June 15). Prognostic Factors of Childhood Medulloblastoma. In Encyclopedia. https://encyclopedia.pub/entry/45635
Ntenti, Charikleia, et al. "Prognostic Factors of Childhood Medulloblastoma." Encyclopedia. Web. 15 June, 2023.
Prognostic Factors of Childhood Medulloblastoma
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Medulloblastomas, highly aggressive neoplasms of the central nervous system (CNS) that present significant heterogeneity in clinical presentation, disease course, and treatment outcomes, are common in childhood. Moreover, patients who survive may be diagnosed with subsequent malignancies during their life or could develop treatment-related medical conditions. 

medulloblastoma tumor histologic pediatrics

1. Introduction

Medulloblastomas (MBs) are highly aggressive neoplasms of the central nervous system (CNS) and are considered the most common malignant brain tumor in childhood [1][2]. First described by Cushing and Bailey in 1925, these tumors derive from discrete neuronal lineages based on molecularly defined subgroups, as shown by recent studies. For example, the cells of origin for the SHH subgroup are the granule-neuron progenitors, whereas for Groups 3 and 4, early rhombic lip is considered the common source of origin [3][4][5].
Epidemiologically, the estimated annual incidence of the tumor in the US is approximately 500 cases per year and the median age of diagnosis is 6–8 years; it is very rarely diagnosed in adults [6].
Since 1990, when the estimated event-free survival (EFS) of MBs was 20–50% [7], and with the application of newer therapeutic techniques, the median overall survival of all subtypes is estimated to be 70% [8][9][10]. The tumor presents a significant biological heterogeneity, as it has been observed that about 30% of patients will be diagnosed with metastatic disease at presentation, and patients who survive may be diagnosed with subsequent malignancies during their life or develop treatment-related neurocognitive, endocrinological, or development disorders, highlighting the need for risk stratification of patients with MB. The aim of risk stratification is the appropriate selection and management of patients who can really benefit from treatment or who need treatment intensification. Clinical and histological factors were the initial determinants of prognosis and the main parameters used for patient categorization into standard and high risk [11][12]. However, the development of new molecular techniques has revolutionized the prognostication of MBs, creating new molecular subgroups with distinct clinical features, response to treatment options, and prognosis. Around 2010, several researchers reported that medulloblastomas comprise at least four distinct molecular subgroups: wingless signaling activated (WNT), Sonic-hedgehog signaling activated (SHH), Group 3, and Group 4, largely based on transcriptome profiles and a few known genetic alterations [13][14][15]. Thereafter, for the first time in 2016, molecular subgroups of MBs were incorporated into the WHO’s MB stratification [16][17]. The WNT subgroup accounts for approximately 10% of all MBs, whereas the SHH subgroup is most common in infants and young adults, accounting for 25% of all MBs [15][18]. Group 3 and Group 4 constitute approximately 65% of medulloblastoma cases and are characterized by great heterogeneity in clinical phenotypes and survival rates [19][20]. The most recent edition of CNS tumor classification (CNS 5), from 2021, divided medulloblastomas into “molecularly” and “histologically” defined, denoting the diverse biology of the tumor [21].
However, although recent research has mainly focused on a thorough investigation of molecular mechanisms behind MB pathogenesis and their contribution to risk stratification of patients, clinicopathological characteristics are also important factors for both relapse and prognosis and have been involved in recently developed prognostic nomograms [22].

2. Clinical and Histological Prognostic Factors

Before the establishment of molecular subgroups of MBs, patient risk stratification was mainly carried out based on their clinical characteristics, the histological features of the tumor, and the treatment approaches followed. Using the above factors as prognostic parameters, patients are categorized into two discrete subtypes: standard and high risk. Age < 3 years old, residual tumor > 1.5 cm2, and large-cell/anaplastic histology are considered high-risk features, whereas patients not fulfilling the above criteria are considered standard risk [9][12][23].

3. Age

One of the clinical characteristics used for risk stratification is the age of the patient. In general, younger age at diagnosis seem to have a worse prognosis compared to older children and is considered a high-risk feature [24]. More precisely, infants and children < 3 years showed the worst prognosis, and various studies have investigated survival rates of those patients [25]. Rutkowski et al. reported a hazard ratio (HR) of 4.02 (95% CI 1.28–12.96) for higher risk for relapse or death for children below 2 years of age compared to children 2–3 years old, whereas results from a Canadian study concluded that children older than 18 months had better survival rates compared to infants below that threshold [26][27]. A possible explanation could be the avoidance of craniospinal irradiation of the tumor in that age group due to the defects that radiotherapy cause in the developing brain. Despite that, a recently published meta-analysis and a retrospective study from Brazil did not reach a statistical significance for age as a poor prognostic factor [8][28][29]. On the other hand, MBs in adults demonstrate a lower mutational rate, are less aggressive, and are associated with better prognosis compared to younger patients [30].

4. Extent of Disease

Another significant factor that affects the prognosis of childhood MBs and is included in the risk-stratification system is the extent of disease. Chang‘s staging is the main clinical classification system for MB patients and evaluates the local extent (T stage) and the tumor’s dissemination (M stage) [31][32]. Although the role of the local extent as a prognostic factor is debated, with some studies showing worse prognosis for tumors located in the midline or with involvement of the fourth ventricle and the brainstem [26][33][34][35] and others not demonstrating similar findings [36][37], the contribution of metastasis to prognosis is well established. It is estimated that 30% of patients present with metastatic disease at diagnosis, which is accompanied by significantly diminished survival rates, with an eight-year OS of 65% and 27% in non-metastatic and metastatic patients, respectively [26]. Special consideration is given to stage M1 (tumor cells disseminated to CFS), which exhibits decreased OS similar to other metastatic stages, implying the need for prompt diagnosis of M1 staging, incorporation into high-risk features, and the implementation of intensifying treatment plans [24][26][38][39].

5. Extent of Resection

Surgical resection comprises an important part of MB treatment plans, and the extent of resection poses a significant prognostic factor. The main goal of resection is the removal of the whole extent of the tumor, with gross total resection (GTR) demonstrating a favorable impact on both PFS and OS, as shown by observational studies and meta-analyses [8][26][34][40]. On the other hand, residual disease after surgery, and especially residual tumors > 1.5 cm2, is considered a poor prognostic factor, leading to local tumor relapses and worst survival rates. However, the benefit of extensive surgical resections may be counterbalanced for post-surgical neurologically adverse events, ranging from endocrinologic abnormalities to neurologic complications such as cerebellar-mutism syndrome, which are present in a considerable percentage of patients after GTR [41]. The latter is of paramount importance mainly for centrally located tumors, in whom total resection might not be feasible and which are categorized as high-risk tumors with a need for treatment intensification. Despite that, Thompson et al., when examining the prognostic value of GTR compared to near-total resection (NTR), along with the integration of clinical and molecular factors, did not conclude significant survival differences, and similar results were also shown by other studies [42][43][44]. Consequently, GTR demonstrates a favorable impact on the prognosis of MB patients, but the EOR should be evaluated in combination with other parameters, such as tumor location and post-surgical complications.

6. Histological Variant

There are four main histological subtypes of MBs: desmoplastic/nodular (DMN), classic (CMB), MBs with extensive nodularity (MBEN), and large cell/anaplastic (LCA). Each of them is characterized by different histological patterns and is associated with distinct molecular and genetic alterations, exhibiting diverse prognosis [3][45][46]. Multiple studies have examined the role of histology as a prognostic factor of MBs. Firstly, DMN, which is characterized by desmoplasia with pericellular fibrinogen deposits, demonstrates better prognosis compared to the classic subtype ([8], desmoplastic vs. classic, HR 0.41, 95% CI 0.31–0.56, OS, and [26], DMN/MBEN vs. classic, HR 0.44, 95% CI 0.31–0.64). Similarly, MBEN histology, which is mainly found in the early years of life, exhibits and good to excellent prognosis [47]. The above survival advantage seems to be maintained even in the presence of adverse prognostic factors (i.e., metastatic setting). In a more detailed way, Leary et al. suggested a similar EFS in non-metastatic compared to metastatic DMN, whereas Gupta et al. showed a better OS in extracranial metastatic desmoplastic compared to non-desmoplastic subtypes [48][49]. On the other hand, LCA, which is characterized by the presence of anaplasia in histopathology, exhibits the worst prognosis compared to other subtypes [46]. Semantically, the degree of anaplasia seems to affect prognosis in a significant manner, where severe anaplasia leads to worse OS and EFS compared to mild. The poor prognosis of that subtype could be attributed to the association of LCA with high-risk features, as it usually affects patients at a younger age, is diagnosed with metastasis at presentation, and is associated with specific molecular and genetic alternations such as LOH, isochromosome 17q, and MYC-family genes [50][51][52]. Especially for the latter, Ellison et al. and Ryan et al. demonstrated the co-expression of c-MYC amplification with severe anaplasia and high-risk features, implying a worse prognosis [53][54].
Despite that, the latest WHO classification combines all the above histological subtypes into one category, called histologically defined MBs, which are associated with specific molecular pathways, suggesting the need for a multilayered evaluation of specific tumor characteristics.

7. Other Prognostic Factors

Except for the above-described clinicopathological and molecular factors, hematological and serum markers are also described in the literature, which can predict the survival of MB patients. Li et al. investigated the role of serum markers on prognosis and revealed that an elevated preoperative neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocyte ratio (PLR) were detected more frequently in Group 3 and Group 4 MBs and were associated independently with worse PFS and OS [55]. In addition, lower levels of lymphocytes during radiotherapy (RT) were associated with increased risk of recurrence [56]. In agreement with this, Zhu et al. evaluated the prognostic value of a systemic inflammatory index (SII) and nutritional status along with serum markers and advocated that high levels of inflammatory markers impaired OS in multivariable models [57].
Apart from serum markers, recent research has focused on the fast-growing field of radiomics, where the incorporation of image analysis into risk-prediction models, which are based on well-established factors, could evaluate the prognosis of each patient preoperatively and at diagnosis. The latter is of paramount importance, as it would assist with further understanding of the diverse nature of MBs and the designation of appropriate treatment strategies. Until recently, radiomic analysis had managed to indirectly predict the prognosis of patients, as MRI findings were associated with the molecular subgroup of the tumor [58][59] or the dissemination to the CSF [60]. However, the co-estimation of imaging findings, clinical characteristics, and molecular subgroup led to the development of nomograms, predicting both PFS [61] and OS [62].

References

  1. Smoll, N.R.; Drummond, K.J. The incidence of medulloblastomas and primitive neurectodermal tumours in adults and children. J. Clin. Neurosci. 2012, 19, 1541–1544.
  2. Salari, N.; Ghasemi, H.; Fatahian, R.; Mansouri, K.; Dokaneheifard, S.; Shiri, M.H.; Hemmati, M.; Mohammadi, M. The global prevalence of primary central nervous system tumors: A systematic review and meta-analysis. Eur. J. Med. Res. 2023, 28, 39.
  3. Massimino, M.; Biassoni, V.; Gandola, L.; Garrè, M.L.; Gatta, G.; Giangaspero, F.; Poggi, G.; Rutkowski, S. Childhood medulloblastoma. Crit. Rev. Oncol. Hematol. 2016, 105, 35–51.
  4. Millard, N.E.; De Braganca, K.C. Medulloblastoma. J. Child Neurol. 2016, 31, 1341–1353.
  5. Smith, K.S.; Bihannic, L.; Gudenas, B.L.; Haldipur, P.; Tao, R.; Gao, Q.; Li, Y.; Aldinger, K.A.; Iskusnykh, I.Y.; Chizhikov, V.V.; et al. Unified rhombic lip origins of group 3 and group 4 medulloblastoma. Nature 2022, 609, 1012–1020.
  6. Brandes, A.A.; Paris, M.K. Review of the prognostic factors in medulloblastoma of children and adults. Crit. Rev. Oncol. Hematol. 2004, 50, 121–128.
  7. Von Bueren, A.O.; Kortmann, R.D.; von Hoff, K.; Friedrich, C.; Mynarek, M.; Müller, K.; Goschzik, T.; Zur Mühlen, A.; Gerber, N.; Warmuth-Metz, M.; et al. Treatment of Children and Adolescents With Metastatic Medulloblastoma and Prognostic Relevance of Clinical and Biologic Parameters. J. Clin. Oncol. 2016, 34, 4151–4160.
  8. Liu, Y.; Xiao, B.; Li, S.; Liu, J. Risk Factors for Survival in Patients With Medulloblastoma: A Systematic Review and Meta-Analysis. Front. Oncol. 2022, 12, 827054.
  9. Hennika, T.; Gururangan, S. Childhood medulloblastoma: Current and future treatment strategies. Expert. Opin. Orphan Drugs 2015, 3, 1299–1317.
  10. Sursal, T.; Ronecker, J.S.; Dicpinigaitis, A.J.; Mohan, A.L.; Tobias, M.E.; Gandhi, C.D.; Jhanwar-Uniyal, M. Molecular Stratification of Medulloblastoma: Clinical Outcomes and Therapeutic Interventions. Anticancer Res. 2022, 42, 2225–2239.
  11. Tarbell, N.J.; Friedman, H.; Polkinghorn, W.R.; Yock, T.; Zhou, T.; Chen, Z.; Burger, P.; Barnes, P.; Kun, L. High-risk medulloblastoma: A pediatric oncology group randomized trial of chemotherapy before or after radiation therapy (POG 9031). J. Clin. Oncol. 2013, 31, 2936–2941.
  12. Gilbertson, R.; Wickramasinghe, C.; Hernan, R.; Balaji, V.; Hunt, D.; Jones-Wallace, D.; Crolla, J.; Perry, R.; Lunec, J.; Pearson, A.; et al. Clinical and molecular stratification of disease risk in medulloblastoma. Br. J. Cancer 2001, 85, 705–712.
  13. Kool, M.; Korshunov, A.; Remke, M.; Jones, D.T.; Schlanstein, M.; Northcott, P.A.; Cho, Y.J.; Koster, J.; Schouten-van Meeteren, A.; van Vuurden, D.; et al. Molecular subgroups of medulloblastoma: An international meta-analysis of transcriptome, genetic aberrations, and clinical data of WNT, SHH, Group 3, and Group 4 medulloblastomas. Acta Neuropathol. 2012, 123, 473–484.
  14. Kool, M.; Koster, J.; Bunt, J.; Hasselt, N.E.; Lakeman, A.; Van Sluis, P.; Troost, D.; Meeteren, N.S.-V.; Caron, H.N.; Cloos, J.; et al. Integrated Genomics Identifies Five Medulloblastoma Subtypes with Distinct Genetic Profiles, Pathway Signatures and Clinicopathological Features. PLoS ONE 2008, 3, e3088.
  15. Gibson, P.; Tong, Y.; Robinson, G.; Thompson, M.C.; Currle, D.S.; Eden, C.; Kranenburg, T.A.; Hogg, T.; Poppleton, H.; Martin, J.; et al. Subtypes of medulloblastoma have distinct developmental origins. Nature 2010, 468, 1095–1099.
  16. Sharma, T.; Schwalbe, E.C.; Williamson, D.; Sill, M.; Hovestadt, V.; Mynarek, M.; Rutkowski, S.; Robinson, G.W.; Gajjar, A.; Cavalli, F.; et al. Second-generation molecular subgrouping of medulloblastoma: An international meta-analysis of Group 3 and Group 4 subtypes. Acta Neuropathol. 2019, 138, 309–326.
  17. Louis, D.N.; Perry, A.; Reifenberger, G.; von Deimling, A.; Figarella-Branger, D.; Cavenee, W.K.; Ohgaki, H.; Wiestler, O.D.; Kleihues, P.; Ellison, D.W. The 2016 World Health Organization Classification of Tumors of the Central Nervous System: A summary. Acta Neuropathol. 2016, 131, 803–820.
  18. Perreault, S.; Ramaswamy, V.; Achrol, A.S.; Chao, K.; Liu, T.T.; Shih, D.; Remke, M.; Schubert, S.; Bouffet, E.; Fisher, P.G.; et al. MRI surrogates for molecular subgroups of medulloblastoma. AJNR Am. J. Neuroradiol. 2014, 35, 1263–1269.
  19. Williamson, D.; Schwalbe, E.C.; Hicks, D.; Aldinger, K.A.; Lindsey, J.C.; Crosier, S.; Richardson, S.; Goddard, J.; Hill, R.M.; Castle, J.; et al. Medulloblastoma group 3 and 4 tumors comprise a clinically and biologically significant expression continuum reflecting human cerebellar development. Cell Rep. 2022, 40, 111162.
  20. Northcott, P.A.; Shih, D.J.H.; Peacock, J.; Garzia, L.; Sorana Morrissy, A.; Zichner, T.; Stütz, A.M.; Korshunov, A.; Reimand, J.; Schumacher, S.E.; et al. Subgroup-specific structural variation across 1000 medulloblastoma genomes. Nature 2012, 488, 49–56.
  21. Louis, D.N.; Perry, A.; Wesseling, P.; Brat, D.J.; Cree, I.A.; Figarella-Branger, D.; Hawkins, C.; Ng, H.K.; Pfister, S.M.; Reifenberger, G.; et al. The 2021 WHO Classification of Tumors of the Central Nervous System: A summary. Neuro Oncol. 2021, 23, 1231–1251.
  22. Guo, C.; Yao, D.; Lin, X.; Huang, H.; Zhang, J.; Lin, F.; Mou, Y.; Yang, Q. External Validation of a Nomogram and Risk Grouping System for Predicting Individual Prognosis of Patients With Medulloblastoma. Front. Pharm. 2020, 11, 590348.
  23. Borowska, A.; Jóźwiak, J. Medulloblastoma: Molecular pathways and histopathological classification. Arch. Med. Sci. 2016, 12, 659–666.
  24. Hagel, C.; Sloman, V.; Mynarek, M.; Petrasch, K.; Obrecht, D.; Kühl, J.; Deinlein, F.; Schmid, R.; von Bueren, A.O.; Friedrich, C.; et al. Refining M1 stage in medulloblastoma: Criteria for cerebrospinal fluid cytology and implications for improved risk stratification from the HIT-2000 trial. Eur. J. Cancer 2022, 164, 30–38.
  25. Lafay-Cousin, L.; Smith, A.; Chi, S.N.; Wells, E.; Madden, J.; Margol, A.; Ramaswamy, V.; Finlay, J.; Taylor, M.D.; Dhall, G.; et al. Clinical, Pathological, and Molecular Characterization of Infant Medulloblastomas Treated with Sequential High-Dose Chemotherapy. Pediatr. Blood Cancer 2016, 63, 1527–1534.
  26. Rutkowski, S.; von Hoff, K.; Emser, A.; Zwiener, I.; Pietsch, T.; Figarella-Branger, D.; Giangaspero, F.; Ellison, D.W.; Garre, M.L.; Biassoni, V.; et al. Survival and prognostic factors of early childhood medulloblastoma: An international meta-analysis. J. Clin. Oncol. 2010, 28, 4961–4968.
  27. Johnston, D.L.; Keene, D.; Bartels, U.; Carret, A.S.; Crooks, B.; Eisenstat, D.D.; Fryer, C.; Lafay-Cousin, L.; Larouche, V.; Moghrabi, A.; et al. Medulloblastoma in children under the age of three years: A retrospective Canadian review. J. Neurooncol. 2009, 94, 51–56.
  28. Bleil, C.B.; Bizzi, J.W.J.; Bedin, A.; de Oliveira, F.H.; Antunes, Á.C.M. Survival and prognostic factors in childhood medulloblastoma: A Brazilian single center experience from 1995 to 2016. Surg. Neurol. Int. 2019, 10, 120.
  29. Walter, A.W.; Mulhern, R.K.; Gajjar, A.; Heideman, R.L.; Reardon, D.; Sanford, R.A.; Xiong, X.; Kun, L.E. Survival and neurodevelopmental outcome of young children with medulloblastoma at St Jude Children’s Research Hospital. J. Clin. Oncol. 1999, 17, 3720–3728.
  30. Franceschi, E.; Giannini, C.; Furtner, J.; Pajtler, K.W.; Asioli, S.; Guzman, R.; Seidel, C.; Gatto, L.; Hau, P. Adult Medulloblastoma: Updates on Current Management and Future Perspectives. Cancers 2022, 14, 3708.
  31. Dufour, C.; Beaugrand, A.; Pizer, B.; Micheli, J.; Aubelle, M.S.; Fourcade, A.; Couanet, D.; Laplanche, A.; Kalifa, C.; Grill, J. Metastatic Medulloblastoma in Childhood: Chang’s Classification Revisited. Int. J. Surg. Oncol. 2012, 2012, 245385.
  32. Chang, C.H.; Housepian, E.M.; Herbert, C., Jr. An operative staging system and a megavoltage radiotherapeutic technic for cerebellar medulloblastomas. Radiology 1969, 93, 1351–1359.
  33. Jiang, T.; Zhang, Y.; Wang, J.; Du, J.; Ma, Z.; Li, C.; Liu, R.; Zhang, Y. Impact of tumor location and fourth ventricle infiltration in medulloblastoma. Acta Neurochir. 2016, 158, 1187–1195.
  34. Zeltzer, P.M.; Boyett, J.M.; Finlay, J.L.; Albright, A.L.; Rorke, L.B.; Milstein, J.M.; Allen, J.C.; Stevens, K.R.; Stanley, P.; Li, H.; et al. Metastasis stage, adjuvant treatment, and residual tumor are prognostic factors for medulloblastoma in children: Conclusions from the Children’s Cancer Group 921 randomized phase III study. J. Clin. Oncol. 1999, 17, 832–845.
  35. Qin, Q.; Huang, D.; Jiang, Y. Survival difference between brainstem and cerebellum medulloblastoma: The surveillance, epidemiology, and end results-based study. Medicine 2020, 99, e22366.
  36. Kumar, L.P.; Deepa, S.F.; Moinca, I.; Suresh, P.; Naidu, K.V. Medulloblastoma: A common pediatric tumor: Prognostic factors and predictors of outcome. Asian J. Neurosurg. 2015, 10, 50.
  37. Nalita, N.; Ratanalert, S.; Kanjanapradit, K.; Chotsampancharoen, T.; Tunthanathip, T. Survival and Prognostic Factors in Pediatric Patients with Medulloblastoma in Southern Thailand. J. Pediatr. Neurosci. 2018, 13, 150–157.
  38. Sanders, R.P.; Onar, A.; Boyett, J.M.; Broniscer, A.; Morris, E.B.; Qaddoumi, I.; Armstrong, G.T.; Boop, F.A.; Sanford, R.A.; Kun, L.E.; et al. M1 Medulloblastoma: High risk at any age. J. Neurooncol. 2008, 90, 351–355.
  39. Hoff, K.V.; Hinkes, B.; Gerber, N.U.; Deinlein, F.; Mittler, U.; Urban, C.; Benesch, M.; Warmuth-Metz, M.; Soerensen, N.; Zwiener, I.; et al. Long-term outcome and clinical prognostic factors in children with medulloblastoma treated in the prospective randomised multicentre trial HIT’91. Eur. J. Cancer 2009, 45, 1209–1217.
  40. Dietzsch, S.; Placzek, F.; Pietschmann, K.; von Bueren, A.O.; Matuschek, C.; Glück, A.; Guckenberger, M.; Budach, V.; Welzel, J.; Pöttgen, C.; et al. Evaluation of Prognostic Factors and Role of Participation in a Randomized Trial or a Prospective Registry in Pediatric and Adolescent Nonmetastatic Medulloblastoma—A Report From the HIT 2000 Trial. Adv. Radiat. Oncol. 2020, 5, 1158–1169.
  41. Ramaswamy, V.; Remke, M.; Bouffet, E.; Bailey, S.; Clifford, S.C.; Doz, F.; Kool, M.; Dufour, C.; Vassal, G.; Milde, T.; et al. Risk stratification of childhood medulloblastoma in the molecular era: The current consensus. Acta Neuropathol. 2016, 131, 821–831.
  42. Thompson, E.M.; Bramall, A.; Herndon, J.E., 2nd; Taylor, M.D.; Ramaswamy, V. The clinical importance of medulloblastoma extent of resection: A systematic review. J. Neurooncol. 2018, 139, 523–539.
  43. Thompson, E.M.; Hielscher, T.; Bouffet, E.; Remke, M.; Luu, B.; Gururangan, S.; McLendon, R.E.; Bigner, D.D.; Lipp, E.S.; Perreault, S.; et al. Prognostic value of medulloblastoma extent of resection after accounting for molecular subgroup: A retrospective integrated clinical and molecular analysis. Lancet Oncol. 2016, 17, 484–495.
  44. Sedano, P.; Segundo, C.G.; De Ingunza, L.; Cuesta-Álvaro, P.; Pérez-Somarriba, M.; Diaz-Gutiérrez, F.; Colino, C.G.; Lassaletta, A. Real-world data for pediatric medulloblastoma: Can we improve outcomes? Eur. J. Pediatr. 2021, 180, 127–136.
  45. Massimino, M.; Antonelli, M.; Gandola, L.; Miceli, R.; Pollo, B.; Biassoni, V.; Schiavello, E.; Buttarelli, F.R.; Spreafico, F.; Collini, P.; et al. Histological variants of medulloblastoma are the most powerful clinical prognostic indicators. Pediatr. Blood Cancer 2013, 60, 210–216.
  46. Orr, B.A. Pathology, diagnostics, and classification of medulloblastoma. Brain Pathol. 2020, 30, 664–678.
  47. Korshunov, A.; Sahm, F.; Stichel, D.; Schrimpf, D.; Ryzhova, M.; Zheludkova, O.; Golanov, A.; Lichter, P.; Jones, D.T.W.; von Deimling, A.; et al. Molecular characterization of medulloblastomas with extensive nodularity (MBEN). Acta Neuropathol. 2018, 136, 303–313.
  48. Leary, S.E.; Zhou, T.; Holmes, E.; Geyer, J.R.; Miller, D.C. Histology predicts a favorable outcome in young children with desmoplastic medulloblastoma: A report from the children’s oncology group. Cancer 2011, 117, 3262–3267.
  49. Gupta, T.; Dasgupta, A.; Epari, S.; Shirsat, N.; Chinnaswamy, G.; Jalali, R. Extraneuraxial metastases in medulloblastoma: Is histology and molecular biology important? J. Neurooncol. 2017, 135, 419–421.
  50. Roussel, M.F.; Robinson, G.W. Role of MYC in Medulloblastoma. Cold Spring Harb. Perspect. Med. 2013, 3, a014308.
  51. von Hoff, K.; Hartmann, W.; von Bueren, A.O.; Gerber, N.U.; Grotzer, M.A.; Pietsch, T.; Rutkowski, S. Large cell/anaplastic medulloblastoma: Outcome according to myc status, histopathological, and clinical risk factors. Pediatr. Blood Cancer 2010, 54, 369–376.
  52. Eberhart, C.G.; Kratz, J.; Wang, Y.; Summers, K.; Stearns, D.; Cohen, K.; Dang, C.V.; Burger, P.C. Histopathological and molecular prognostic markers in medulloblastoma: C-myc, N-myc, TrkC, and anaplasia. J. Neuropathol. Exp. Neurol. 2004, 63, 441–449.
  53. Ellison, D.W.; Kocak, M.; Dalton, J.; Megahed, H.; Lusher, M.E.; Ryan, S.L.; Zhao, W.; Nicholson, S.L.; Taylor, R.E.; Bailey, S.; et al. Definition of disease-risk stratification groups in childhood medulloblastoma using combined clinical, pathologic, and molecular variables. J. Clin. Oncol. 2011, 29, 1400–1407.
  54. Ryan, S.L.; Schwalbe, E.C.; Cole, M.; Lu, Y.; Lusher, M.E.; Megahed, H.; O’Toole, K.; Nicholson, S.L.; Bognar, L.; Garami, M.; et al. MYC family amplification and clinical risk-factors interact to predict an extremely poor prognosis in childhood medulloblastoma. Acta Neuropathol. 2012, 123, 501–513.
  55. Li, K.; Duan, W.-C.; Zhao, H.-B.; Wang, L.; Wang, W.-W.; Zhan, Y.-B.; Sun, T.; Zhang, F.-J.; Yu, B.; Bai, Y.-H.; et al. Preoperative Neutrophil to Lymphocyte Ratio and Platelet to Lymphocyte Ratio are Associated with the Prognosis of Group 3 and Group 4 Medulloblastoma. Sci. Rep. 2019, 9, 13239.
  56. Grassberger, C.; Shinnick, D.; Yeap, B.Y.; Tracy, M.; Ellsworth, S.G.; Hess, C.B.; Weyman, E.A.; Gallotto, S.L.; Lawell, M.P.; Bajaj, B.; et al. Circulating Lymphocyte Counts Early During Radiation Therapy Are Associated With Recurrence in Pediatric Medulloblastoma. Int. J. Radiat. Oncol. Biol. Phys. 2021, 110, 1044–1052.
  57. Zhu, S.; Cheng, Z.; Hu, Y.; Chen, Z.; Zhang, J.; Ke, C.; Yang, Q.; Lin, F.; Chen, Y.; Wang, J. Prognostic Value of the Systemic Immune-Inflammation Index and Prognostic Nutritional Index in Patients With Medulloblastoma Undergoing Surgical Resection. Front. Nutr. 2021, 8, 754958.
  58. Dasgupta, A.; Gupta, T.; Pungavkar, S.; Shirsat, N.; Epari, S.; Chinnaswamy, G.; Mahajan, A.; Janu, A.; Moiyadi, A.; Kannan, S.; et al. Nomograms based on preoperative multiparametric magnetic resonance imaging for prediction of molecular subgrouping in medulloblastoma: Results from a radiogenomics study of 111 patients. Neuro Oncol. 2019, 21, 115–124.
  59. Iv, M.; Zhou, M.; Shpanskaya, K.; Perreault, S.; Wang, Z.; Tranvinh, E.; Lanzman, B.; Vajapeyam, S.; Vitanza, N.A.; Fisher, P.G.; et al. MR Imaging-Based Radiomic Signatures of Distinct Molecular Subgroups of Medulloblastoma. AJNR Am. J. Neuroradiol. 2019, 40, 154–161.
  60. Zheng, H.; Li, J.; Liu, H.; Wu, C.; Gui, T.; Liu, M.; Zhang, Y.; Duan, S.; Li, Y.; Wang, D. Clinical-MRI radiomics enables the prediction of preoperative cerebral spinal fluid dissemination in children with medulloblastoma. World J. Surg. Oncol. 2021, 19, 134.
  61. Liu, Z.M.; Zhang, H.; Ge, M.; Hao, X.L.; An, X.; Tian, Y.J. Radiomics signature for the prediction of progression-free survival and radiotherapeutic benefits in pediatric medulloblastoma. Childs Nerv. Syst. 2022, 38, 1085–1094.
  62. Yan, J.; Zhang, S.; Li, K.K.; Wang, W.; Li, K.; Duan, W.; Yuan, B.; Wang, L.; Liu, L.; Zhan, Y.; et al. Incremental prognostic value and underlying biological pathways of radiomics patterns in medulloblastoma. EBioMedicine 2020, 61, 103093.
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