Adult's Medulloblastoma



Abstract Views
386

Downloads
178

Citations

Share

##plugins.themes.bootstrap3.article.sidebar##

Submitted Jul 11, 2022
Published Oct 23, 2022
10.24018/ejmed.2022.4.5.1418

Abstract viewed = 386 times

Table of Contents

##plugins.themes.bootstrap3.article.main##

Background and Objectives: Medulloblastoma are a highly malignant tumor of the central nervous system, very frequent in the childhood, but remains rare in adulthood. This study aims to analyze the frequency, clinical and therapeutic aspects of adults medulloblastomas.

Methods: We retrospectively analyzed the data from the record of adult’s patients treated for medulloblastoma between between January 2000 and December 2015 in in the Radiation oncology department of the Ibn Rochd University Hospital Center.

Results: The average age was 27,96 years (Range: 20-53 years). The sex ratio M/F was 4 with a clear male predominance. The most common symptom was intracranial hypertension, which was present in 22 patients, followed by headaches and cerebellar syndrome which were present in 9 patients. The tumor was mostly located in the cerebellar hemispheres with an average size of 5,75 cm. One patient had pulmonary metastasis at the time of diagnosis.

Twenty-four patients had a surgical resection. Complete resection was performed in 13 patients. Thus, twenty-one patients have received craniospinal irradiation with a dose of 36 Gy followed by a boost in the posterior fossa with a total dose of 54-56 Gy. Twelve patients have received Chemotherapy. Six patients are still alive. Survival rates were 50% at 3 years, 35% at 5 years, and 30% at 10 years.

Conclusion: Medulloblastoma is a devastating disease with a bad prognosis. The clinical presentation is variable and the management is multidisciplinary. Radiotherapy treatment with or without Chemotherapy play an important role in the control of the disease and recurrences avoidance. Our study illustrates the importance of treatment by radiation.

Keywords: Adults medulloblastoma, craniospinal irradiation, radiotherapy

Downloads

Download data is not yet available.

References

  1. Taillandier L, Blonski M, Carrie C, Bernier V, Bonnetain F, Bourdeaut F, et al. Les médulloblastomes: revue générale. Revue Neurologique. 2011; 167(5): 431-48. French.
    DOI  |   Google Scholar
  2. Ostrom QT, Gittleman H, Liao P, Rouse C, Chen Y, Dowling J, et al. CBTRUS statistical report: primary brain and central nervous system tumors diagnosed in the united states in 2007-2011. Neuro-Oncology. 2014; 16(suppl 4): iv1–iv63.
    DOI  |   Google Scholar
  3. Gaviani P, Simonetti G, Rudà R, Franchino F, Lombardi G, Possanzini M, et al. Medulloblastoma of the adult: results from a multicenter retrospective study by Aino (italian association of neuro-oncology) and Sin (italian society of neurology). Neurological Sciences. 2020; 42(2): 665–71.
    DOI  |   Google Scholar
  4. Rorke LB. The cerebellar medulloblastoma and its relationship to primitive neuroectodermal tumors. J Neuropathol Exp Neurol. 1983; 42(1): 1-15.
    DOI  |   Google Scholar
  5. Wooley JR, Penas-Prado M. Pediatric versus adult medulloblastoma: towards a definition that goes beyond age. Cancers (Basel). 2021; 13(24): 6313.
    DOI  |   Google Scholar
  6. Louis DN, Perry A, Reifenberger G, von Deimling A, Figarella-Branger D, Cavenee WK, et al. The 2016 world Health Organization classification of tumors of the central nervous system: A summary. Acta Neuropathologica. 2016; 131(6): 803–20.
    DOI  |   Google Scholar
  7. Orr BA. Pathology, diagnostics, and classification of medulloblastoma. Brain Pathology. 2020; 30(3): 664–78.
    DOI  |   Google Scholar
  8. Padovani L, Sunyach M-P, Perol D, Mercier C, Alapetite C, Haie-Meder C, et al. Common strategy for adult and pediatric medulloblastoma: A multicenter series of 253 adults. International Journal of Radiation Oncology Biology Physics. 2007; 68(2): 433–40.
    DOI  |   Google Scholar
  9. Chan AW, Tarbell NJ, Black PML, Louis DN, Frosch MP, Ancukiewicz M, et al. Adult medulloblastoma: Prognostic factors and patterns of relapse. Neurosurgery. 2000; 47(3): 623–32.
    DOI  |   Google Scholar
  10. Park TS, Hoffman HJ, Hendrick EB, Humphreys RP, Becker LE. Medulloblastoma: Clinical presentation and management. Journal of Neurosurgery. 1983; 58(4): 543–52.
    DOI  |   Google Scholar
  11. Hovestadt V, Ayrault O, Swartling FJ, Robinson GW, Pfister SM, Northcott PA. Medulloblastomics revisited: Biological and clinical insights from thousands of patients. Nature Reviews Cancer. 2019; 20(1): 42–56.
    DOI  |   Google Scholar
  12. Louis DN, Perry A, Wesseling P, Brat DJ, Cree IA, Figarella-Branger D, et al. The 2021 who classification of tumors of the central nervous system: A summary. Neuro-Oncology. 2021; 23(8): 1231–51.
    DOI  |   Google Scholar
  13. Majd N, Penas-Prado M. Updates on management of adult medulloblastoma. Current Treatment Options in Oncology. 2019; 20(8).
    DOI  |   Google Scholar
  14. Chang CH, Housepian EM, Herbert C. An operative staging system and a megavoltage radiotherapeutic technic for cerebellar medulloblastomas. Radiology. 1969; 93(6): 1351–9.
    DOI  |   Google Scholar
  15. Packer RJ. Medulloblastoma: Clinical and biologic aspects. Neuro-Oncology. 1999; 1(3): 232–50.
    DOI  |   Google Scholar
  16. Thompson EM, Hielscher T, Bouffet E, Remke M, Luu B, Gururangan 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(4): 484–95.
    DOI  |   Google Scholar
  17. Lai S-F, Wang C-W, Chen Y-H, Lan K-H, Cheng JC-H, Cheng A-L, et al. Medulloblastoma in adults. Strahlentherapie und Onkologie. 2012; 188(10): 878–86.
    DOI  |   Google Scholar
  18. Northcott PA, Buchhalter I, Morrissy AS, Hovestadt V, Weischenfeldt J, Ehrenberger T, et al. The whole-genome landscape of medulloblastoma subtypes. Nature. 2017; 547(7663): 311–7.
    DOI  |   Google Scholar
  19. Schwalbe EC, Lindsey JC, Nakjang S, Crosier S, Smith AJ, Hicks D, et al. Novel molecular subgroups for clinical classification and outcome prediction in childhood medulloblastoma: a cohort study. Lancet Oncol. 2017; 18(7): 958–71.
    DOI  |   Google Scholar
  20. Paterson E, Farr RF. Cerebellar medulloblastoma: treatment by irradiation of the whole central nervous system. Acta Radiol. 1953; 39(4): 323–36.
    DOI  |   Google Scholar
  21. Lannering B, Rutkowski S, Doz F, Pizer B, Gustafsson G, Navajas A, et al. Hyperfractionated versus conventional radiotherapy followed by chemotherapy in standard-risk medulloblastoma: results from the randomized multicenter HIT-SIOP PNET 4 trial. J Clin Oncol. 2012; 30(26): 3187–93.
    DOI  |   Google Scholar
  22. Michalski JM, Janss A, Vezina G, Gajjar A, Pollack I, Merchant TE, et al. Results of COG ACNS0331: A phase III trial of involved-field radiotherapy (IFRT) and low dose craniospinal irradiation (LD-CSI) with chemotherapy in average-risk medulloblastoma: A report from the children’s oncology group. Int J Radiat Oncol Biol Phys. 2016; 96(5): 937–8.
    DOI  |   Google Scholar
  23. Ajithkumar T, Horan G, Padovani L, Thorp N, Timmermann B, Alapetite C, et al. SIOPE - Brain tumor group consensus guideline on craniospinal target volume delineation for high-precision radiotherapy. Radiother Oncol. 2018; 128(2): 192–7.
    DOI  |   Google Scholar
  24. Thomas A, Noël G. Medulloblastoma: optimizing care with a multidisciplinary approach. J Multidiscip Healthc. 2019; 12: 335–47.
    DOI  |   Google Scholar
  25. Hau P, Frappaz D, Hovey E, McCabe MG, Pajtler KW, Wiestler B, et al. Development of randomized trials in adults with medulloblastoma-the example of EORTC 1634-BTG/NOA-23. Cancers (Basel). 2021; 13(14): 3451.
    DOI  |   Google Scholar
  26. Seravalli E, Bosman M, Lassen-Ramshad Y, Vestergaard A, Oldenburger F, Visser J, et al. Dosimetric comparison of five different techniques for craniospinal irradiation across 15 European centers: analysis on behalf of the SIOP-E-BTG (radiotherapy working group). Acta Oncol. 2018; 57(9): 1240–9.
    DOI  |   Google Scholar
  27. Baliga S, Gandola L, Timmermann B, Gail H, Padovani L, Janssens GO, et al. Brain tumors: Medulloblastoma, ATRT, ependymoma. Pediatr Blood Cancer. 2021; 68 Suppl 2(S2): e28395.
    DOI  |   Google Scholar
  28. Eaton BR, Esiashvili N, Kim S, Weyman EA, Thornton LT, Mazewski C, et al. Clinical outcomes among children with standard-risk medulloblastoma treated with proton and photon radiation therapy: A comparison of disease control and overall survival. Int J Radiat Oncol Biol Phys. 2016; 94(1): 133–8.
    DOI  |   Google Scholar
  29. Friedrich C, von Bueren AO, von Hoff K, Kwiecien R, Pietsch T, Warmuth-Metz M, et al. Treatment of adult nonmetastatic medulloblastoma patients according to the paediatric HIT 2000 protocol: a prospective observational multicentre study. Eur J Cancer. 2013; 49(4): 893–903.
    DOI  |   Google Scholar
  30. Radiation therapy and combination chemotherapy in treating young patients with medulloblastoma, supratentorial primitive neuroectodermal tumor, or ependymoma. [Internet] 2014. Available from: https://www.clinicaltrials.gov/ct2/show/NCT00303810
     Google Scholar
  31. Packer RJ, Sutton LN, Goldwein JW, Perilongo G, Bunin G, Ryan J, et al. Improved survival with the use of adjuvant chemotherapy in the treatment of medulloblastoma. J Neurosurg. 1991; 74(3): 433–40.
    DOI  |   Google Scholar


Most read articles by the same author(s)

1 2 3 > >>