Adult Medulloblastoma: Clinical Profile and Treatment Result of 25 Patients

Year 2020, , 131 - 138, 19.09.2020

Ali Arslan Vedat Açık

https://doi.org/10.5455/umj.20200712045718

Abstract

Introduction: The study aimed to discuss the clinical presentation of radiological findings, surgical treatments, and postoperative complications as well as the radiotherapy and chemotherapy options for adult patients with medulloblastoma.
Materials and Methods: A total of 25 patients (14 males and 11 females) between the ages of 17 and 63 who underwent an operation at our clinic between 2006-2016 are discussed in this article. The degree of resection in the patients and tumor sizes after radiotherapy (RT) and chemotherapy (Cht) was compared using the patients’ preoperative and postoperative magnetic resonance (MR) images.
Results: The average age of the patients was 30.9 years. Gross total resection was achieved in 20 patients, whereas subtotal resection was achieved in the remaining 5 patients. Three patients developed hydrocephalus during the 6th month after resection, which was treated with a V-P shunt. Another four patients developed a cerebrospinal fluid (CSF) fistula. Among the five patients who only achieved subtotal resection, two died during the 24th month of the post-operative period, and the other three died during the 18th month. Eleven patients presented pathology of the desmoplastic type, and 14 patients presented classical medulloblastoma. Spinal metastasis was detected in three patients during the pre-operative follow-up and in three patients during the post-operative follow-up. The average survival rate of the cohort was 60% during the 3 to 5-year follow-up period.
Conclusion: Medulloblastoma development is a risk not only for children and adolescents but also for adults. Maximum tumor removal during treatment affects patient outcomes, and RT and Cht administration during the post-operative period can extend the life span of these patients.

Keywords

Adult Medulloblastoma, Resection, chemotherapy,

References

• Russell DS, Rubinstein LJ. Pathology of tumors of the nervous system. 5th ed. London: Edward Arnold, 1989
• Katsetos CD, Herman MM, Frankfurter A, Gass P, Collins VP, Walker CC, et al. Cerebellar desmoplastic medulloblastomas. A further immunohistochemical characterization of the reticulin-free pale islands. Arch Pathol Lab Med. 1989;113(9): 1019-1029
• Abacioglu U, Uzel O, Sengoz M, Turkan S, Ober A. Medulloblastoma in adults: treatment results and prognostic factors. Int J Radiat Oncol Biol Phys, 2002;54(3):855–860
• Brandes AA, Paris MK. Review of the prognostic factors in medulloblastoma of children and adults. Crit Rev Oncol Hematol. 2004;50(2):121-128
• Chan AW, Tarbell NJ, Black PM, Louis DN, Frosch MP, Ancukiewicz M, et al. Adult medulloblastoma: prognostic factors and patterns of relapse. Neurosurgery. 2000;47(3), 623-31
• Brandes AA, Ermani M, Amista P, Basso U, Vastola F, Gardiman M, et al. The treatment of adults with medulloblastoma: a prospective study. Int J Radiat Oncol Biol Phys. 2003;57(3):755–761
• Brandes AA, Franceschi E, Tosoni A, Blatt V, Ermani M. Long- term results of a prospective study on the treatment of medulloblastoma in adults. Cancer. 2007;110(9):2035–2041
• 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
• Northcott PA, Hielscher T, Dubuc A, Mack S, Shih D, Remke M, et al. Pediatric and adult sonic hedgehog medullo blastomas are clinically and molecularly distinct. Acta Neuropathol. 2011;122(2):231–240
• Kool M, Korshunov A, Remke M, Jones DT, Schlanstein M, Northcott PA, et al. Molecular subgroups of medullo blastoma: an international meta-analysis of transcriptome, genetic aberrations, and clinical data of WNT, SHH, Group 3, and Group 4 medulloblastomas. Acta Neuropathol. 2012;123(4):473-484
• Remke M, Hielscher T, Northcott PA, Witt H, Ryzhova M, Wittmann A, et al. Adult medulloblastoma comprises three major molecular variants. J Clin Oncol. 2011;29(19):2717–2723
• Birch JM, Marsden HB, Jones PH, Pearson D, Blair V. Improvements in survival from childhood cancer: results of a population based survey over 30 years. Brit Med J. 1988;296 (6633):1372–1376
• Reni M, Gatta G, Vecht et al. Adult neuroectodermal tumors of posterior fossa (medulloblastoma) and of supratentorial sites (stPNET). Crit Rev Oncol Hematol. 2009;71(2):165‑179
• Resenbium MK,Bilbao JM ,Ang LC. Neuromuscular system. In: Ackerman`s surgical pathology. Volume 2, 8th ed. St Louis: Mosby. 1996;p.231-2315
• Taylor MD, Northcott PA, Korshunov A, Remke M, Cho YJ, Clifford SC, et al. Molecular subgroups of medulloblastoma: the current consensus. Acta Neuropathol. 2012;123(4):4
• Bezircioglu H, Sucu HK, Minoglu M, Tunakan M,Olmezoglu A. Adult medulloblastoma: clinical profile and treatment result of 13 patients. J Neurol Sci. 2009;26:(4),463-471.
• Koci TM, Chiang F, Mehringer CM, Yuh WT, Mayr NA, Itabashi H, et al. Adult cerebellar medulloblastoma: imaging features with emphasis on MR findings. AJNR. 1993;14(4), 929-939.
• Riffaud L, Saikali S, Leray E, Hamlat A, Haegelen C, Vauleon E, et al. Survival and prognostic factors in a series of adults with medulloblastomas. J NeuroSurg. 2009;111(3):478-487
• Jiang T, Zhu J, Dong J, Wang JM, Du J, Li CD, et al. Clinical outcomes of adult medulloblastoma: A retrospective analysis at a single institute. Transl Neurosci Clin. 2015;1(1):17–24
• Lai R. Survival of patients with adult medulloblastoma: a population-based study. Cancer. 2008;112(7):1568-1574
• Kocakaya S, Beier CP, Beier D. Chemotherapy increases long-term survival in patients with adult medulloblastoma- rature-based meta-analysis. Neuro Oncol. 2016;18(3):408-416