Padilla-Vázquez F, de Montesinos-Sampedro A, Escobar-de la Garma VH, Mendizábal-Guerra R

Language: Spanish
References: 17
Page: 61-66
PDF size: 201.82 Kb.

ABSTRACT

The atypical teratoid rhabdoid tumor (TA/RT), is a malignant embryonal lession that corresponds to the 1-3% of the central nervous system tumors in children younger than 18 years, but up to 20% of malignant tumors of the CNS in patients younger than 3 years of age, generally are infratentorial, whose prognosis is bad in the short term, with a mortality rate of 70% per year and it increases in patients with metastatic disease at the time of diagnosis, tumor recurrence is 80% per year. The clinical presentation, is highly variable, since it depends to the site where the lesion is located, although in pediatric patients the lesion is on infratentorial region, so generated alternate syndromes, symptoms of injury of cranial nerves, cerebellar symptoms and 33% of these patients have hydrocephalus. On histopathologic examination, cells are seen with a rhabdoid tumor and mesenquima component, in addition to small cells. The immunohistochemistry is essential in these lesions. We have carried out many studies in the last two decades in an attempt to increase the survival in these patients, without much success for the moment, however it has been observed that the most important component in the treatment is radiation therapy since the beginning of this early during the first month of postoperative increases the survival rate, back to this is mandatory start handling of chemotherapy.

REFERENCES

1. Tekautz TM, Fuller CE, Blaney S, Fouladi M, Broniscer A,Merchant TE, et al. Atypical teratoid/rhabdoid tumors (ATRT): improved survival in children 3 years of age and older with radiation therapy and high-dose alkylator-based chemotherapy. J Clin Oncol 2005; 23(7):1491-9.

2. l. han. Atypical Teratoid/Rhabdoid Tumors in adult patients: TC and MR imaging features. AJNR Am J Neuroradiol 32:103-8.

3. Rahmat K. A Child with atypical teratoid/rhabdoid tumour of the posterior cranial fossa. Singapore Med J 2008;49(12): e365.

4. Garré ML, Cama A, Milanaccio C, Gandola L, Massimino M. New concepts in the treatment of brain tumors in very young children. Expert Rev Neurother 2006, 6(4):489-500.

5. Atmaram S. Sequencing of local therapy affects the pattern of treatment failure and survival in children with atypical teratoid rhabdoid tumors of the central nervous system. Int J Radiat Oncol Biol Phys 2012 april 1; 82(5): 1756-63.

6. Eun Sil Park. Tandem high-dose chemotherapy and autologous stem cell transplantation in young children with atypical teratoid/rhabdoid tumor of the central nervous system. J Korean Med Sci 2012; 27: 135-40.

7. Fatma Markoç. Atipik teratoid rabdoid tümör: Olgu Sunumu, Turk Patoloji Derg 2013, 29:160-3.

8. Kevin F. Atypical teratoid rhabdoid tumor: current therapy and future directions, frontiersinoncology. Pediatric Oncology 2012;(2)114.

9. Kin Hup Chan. A case report of atypical teratoid/rhabdoid tumour in a 9-year-old girl. Malaysian J Med Sci 2011;18(3):82-6.

10. Bruggers CS, Bleyl SB, Pysher T, Barnette P, Afify Z, Walker M, et al. Clinicopathologic comparison of familial vs sporadic atypical teratoid/rhabdoid tumors (AT/RT) of the central nervous system. Pediatr Blood Cancer 2011;56(7):1026-31.

11. Piergiorgio Modena. Case report: long-term survival of an infant syndromic patient affected by atypical teratoid-rhabdoid tumor. BMC Cancer 2013, 13:100.

12. Dufour C, Beaugrand A, Le Deley MC, Bourdeaut F, Andre N, Leblond P, et al. Clinico pathologic prognostic factors in childhood atypical teratoid and rhabdoid tumor of the central ner vous system: a multicenter study. Cancer 2012, 118(15):3812-21.

13. Pfister SM, Korshunov A, Kool M, Hasselblatt M, Eberhart C, Taylor. Molecular diagnostics of CNS embryonal tumors. Ac Neuropathol 2010, 120(5):553-66.

14. Biegel JA, Pollack IF. Molecular analysis of pediatric brain tumors. Curr Oncol Rep 2004, 6(6):445-52.

15. Judkins AR, Mauger J, Ht A, Rorke LB, Biegel JA. Immunohistochemical analysis of hSNF5/INI1 in pediatric CNS neoplasms. Am J Surg Pathol 2004, 28(5):644-50.

16. Kordes U, Gesk S, Fruhwald MC, Graf N, Leuschner I, Hasselblatt M, et al. Clinical and molecular features in patients with atypical teratoid rhabdoid tumor or malignant rhabdoid tumor. Genes Chromosomes Cancer 2010;49(2):176-81.

17. Gandola L, Massimino M, Cefalo G, Solero C, Spreafico F, Pecori E, et al. Hyperfractionated accelerated radiotherapy in the Milan strategy for metastatic medulloblastoma. J Clin Oncol 2009, 27(4):566-71.