Castillón-Benavides NK, Salinas-Lara C, Castilleros M, Arellano A, Vega-Orozco R, Rembao-Bojorquez D, Tena-Suck ML

Language: Spanish
References: 31
Page: 217-223
PDF size: 202.84 Kb.

ABSTRACT

Medulloblastomas are brain tumors making up 15 to 25% of all pediatric neoplasias. Recently, the WHO reclassified medulloblastomas into: classical, desmoplastic, nodular or intensively nodular, anaplastic and giant cells. The extensively nodular desmoplastic type is separated from the desmoplastic one, since this type corresponds to a kind with a worse prognosis. Material and methods: at the Neuropathology Department, we reviewed medulloblastomas for a 5-year period and reclassified them according to the new WHO classification. This is a cohort, retrospective, observational study following up cases over a 5 year period. Castillón Benavides, et al. Results: 43 cases of medulloblastoma in adults were identified, from which 17 (39.5%) were the classical type, 12 (27.9%) were desmoplastic with scarce nodules, 4 (9.3%) were extremely nodular and 3 (7%) were anaplastic. The age of the patients studied was between 15 and 59 years (mean 29.16), 29 cases were male (67.4%) and 14 female (32.6%). The symptoms began between the 1st and 36th month (mean 3.73). Location: in cerebellum, 42 (92.7%); and one in the pineal gland (2.3%). On the left side there were 21 cases (48.8%), on the right side 13 cases (30.2%), and in the vermis 9 (29.9%). Thirty six (83.7%) cases received complementary radiotherapy and 6 (14%) chemotherapy. We found improvement in 23 cases (53.5%), no improvement in 7 (16.3%), worsening in 9 (20.9%), and 4 deaths (9.3%). Relapse appeared in 19 cases (42.2%), from which 12 (27.9%), were male and 7 (16.2%), female. Due to histological type: 10 cases (52.6%) were classical type, 7 (36.8%) desmoplasic type with scarce nodules and 1 (5.2%) with abundant nodules and 1 anaplastic type (5.2%)(p=.620). Discussion: reclassifying according to the WHO proposed new classification, specifically to the formation of few or several nodules is related to the prognosis, as isolated cases of desmoplasic medulloblastomas with extensive nodules had been reported as the most aggressive histological subtype. Calling it that way or placing emphasis on nodularity is a factor that must be taken into account in the subsequent treatment of the patient.

REFERENCES

1. Kleihues P, Cavenee WK, Eds. Pathology and genetics of tumors of the nervous system. World Health Organization Classification of tumours. Lyon: IARC Press, 2000.

2. Burger PC, Sheithauer BW. Tumors of the central nervous system. Washington DC: Armed Forces Institute of Pathology (AFIP),1994.

3. Nakano I, Hemmati HD, Kornblum Hl. Cancer stem cell in pediatric brain tumor. No Shinkei Geka 2004; 32: 827-34.

4. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, et al. The 2007 WHO Classification of Tumours of the Central Nervous System. Acta Neuropathol (Berl) 2007;114:97-109.

5. Rodriguez FJ, Eberhart C, O’Neill BP, Slezak J, Burger PC, Goldthwaite P, et al. Histopathologic grading of adult medulloblastomas. Cancer 2007;109:2557-65.

6. McManamy CS, Pears J, Weston CL, Hanzely Z, Ironside JW, Taylor RE, et al. Clinical Brain Tumour Group . Nodule formation and desmoplasia in medulloblastomas-defining the nodular/ desmoplastic variant and its biological behavior. Brain Pathol 2007;17:151-64.

7. Sarkar C, Deb P, Sharma MC. Medulloblastomas: New directions in risk stratification. Neurol India 2006;54:16-23.

8. Sousa R, Sa G, Reimao S, Lopes L, Ruivo J, Albuquerque L, et al. Adult cerebellar medulloblastoma: imaging findings in eight cases Acta Med Port 2006;19:466-70.

9. Fransolet AC, Born JD, Misson JP, Dresse MF, Forget P, Rausin L, et al. Management of medulloblastoma in children: the experience of a single institution in Liege from 1991 to 2005. Rev Med Liege 2007;62:200-4.

10. Rutkowski S, von Bueren A, von Hoff K, Hartmann W, Shalaby T, Deinlein F, et al. Prognostic relevance of clinical and biological risk factors in childhood medulloblastoma: results of patients treated in the prospective multicenter trial HIT’91. Clin Cancer Res 2007;13:2651-7.

11. Polkinghorn WR, Tarbell NJ. Medulloblastoma: tumorigenesis, current clinical paradigm, and efforts to improve risk stratification. Nat Clin Pract Oncol 2007;4:295-304.

12. Vogel H, Fuller GN. Primitive neuroectodermal tumors, embryonal tumors, and other small cell and poorly differentiated malignant neoplasms of the central and peripheral nervous systems. Ann Diagn Pathol 2003;7: 387-98.

13. Ellison D. Classifying the medulloblastoma: insights from morphology and molecular genetics. Neuropathol Appl Neurobiol 2002; 28:257-82.

14. Brandes AA, Paris MK. Review of the prognostic factors in medulloblastoma of children and adults. Crit Rev Oncol Hematol 2004 ;50:121-8.

15. Giordana MT, Cavalla P, Dutto A, Borsotti L, Chio A, Schiffer D. Is medulloblastoma the same tumor in children and adults? J Neurooncol 1997 ;35: 169-76.

16. Giordana MT, Cavalla P, Chio A, Marino S, Soffietti R, Vigliani MC, et al. Prognostic factors in adult medulloblastoma. A clinicopathologic study. Tumori 1995; 81: 338-46.

17. Eberhart CG, Cohen KJ, Tihan T, Goldthwaite PT, Burger PC. Medulloblastomas with systemic metastases: evaluation of tumor and clinical behavior in 23 patients. Histopathology. J Pediatr Hematol Oncol 2003;25: 198-203.

18. Radner H, Blumcke I, Reifenberger G, Wiestler OD. The new WHO classification of tumors of the nervous system 2000. Pathology and Genetics. Pathologe 2002; 23: 260-83.

19. McManamy CS, Lamont JM, Taylor RE, Cole M, Pearson AD, Clifford SC, et al. Morphophenotypic variation predicts clinical behavior in childhood non-desmoplastic medulloblastomas. J Neuropathol Exp Neurol 2003;62: 627-3.

20. Ho DM, Hsu CY, Chiang H. Histopathologic grading of medulloblastomas.

21. Eberhart CG, Burger PC. Anaplasia and grading in medulloblastomas. Brain Pathol 2003; 13: 376-85.

22. Perry A. Medulloblastoma with favorable versus unfavorable histology: how many small blue cell tumor types are there in the brain? Adv Anat Pathol 2002;9:345-50.

23. Brandes AA, Ermani M, Amista P, Basso U, Vastola F, Gardiman M, et al. The treatment of adults with medulloblas-toma: a prospective study. Int J Radiat Oncol Biol Phys 2003; 57: 755-61.

24. Paulino AC.Current multimodality management of medulloblastoma. Curr Probl Cancer 2002; 26: 317-26.

25. Lal P, Nagar YS, Kumar S, Singh S, Maria Das KJ, Narayan SL, et al. Medulloblastomas: clinical profile, treatment techniques and outcome: An institutional experience. Indian J Cancer 2000; 39:97-105.

26. Urberuaga A, Navajas A, Burgos J, Pijoan JI. A review of clinical and histological features of medulloblastomas during the last 21 years. Spanish paediatric. Childs Nery Syst 2006;22:466-74.