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Academia Mexicana de Neurología, A.C.

2013, Number 6

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Rev Mex Neuroci 2013; 14 (6)

Atypical teratoid-rhabdoid tumor: Case reports and literature review

Ramírez-Aguilar R, Escobar GVH, Madrid-Sánchez AJ, Guadarrama-Ortiz P, De Montesinos-Sampedro A, Mendizabal-Guerra R

Language: Spanish
References: 22
Page: 356-360
PDF size: 845.18 Kb.


ABSTRACT

Introduction: Atypical teratoid-rhabdoid tumor (ATRT) is an extremely agressive malignant tumor that accounts for 1-2% of CNS tumors in pediatric patients. Although this entity was classified as a variety of teratomas, it has been confirmed that immunohistochemically there is positivity for vimentin, epithelial-membrane antigen and glial fibrillar acidic protein.
Case Reports: We present two cases of infants with this tumor that were operated with subsecuent chemotherapy and radiotherapy in Hospital Juárez de México. Patient 1: 11-month infant with history of hidrocephalus treated with ventriculoperitoneal shunt, we performed valvular change because of associated disfunction and suboccipital craniectomy. A few days later a supra/infratentorial approach was done with resection of the tumor. He received chemotherapy. The survival was 5 months. Patient 2: 8-year children present in the emergency room with a history of 2 week headache, nausea and vomiting and left hemiparesia; a CT scan showed giant right parietal tumor, few days later a frontoparietotemporal craniectomy was done for partial resection of the tumor, posteriorly he received chemotherapy and radiotherapy.
Conclusion:ATRT is a relatively new entity, with few cases reported in medical literature, extremely aggresive with por survival. We report two different cases with this tumor that were operated, however some tumor features made it difficult to resect them completely, including infiltration to brain parenchyma, tumor size and blood irrigation.


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