Abstract
Objective
The purpose of this study is to present our experience with ten cases of Central nervous system atypical teratoid rhabdoid tumor (CNS/ATRT).
Patients and methods
A series of ten patients with CNS/ATRT, were diagnosed and treated between 1990 and 2005, at the National Institute of Pediatrics, in Mexico City. The gender, age of presentation, clinical features, tumor localization, imaging studies, grade of tumor resection, complications, adjuvant therapy, and survival are presented.
Results
The mean age at diagnosis was 37.8 months, seven cases were male, and their average clinical course was 1.3 months. The more common clinical presentation was intracranial hypertension with cranial nerve deficits; location was infratentorial in four patients and supratentorial in six. Hydrocephalus was present as the most common complication (seven cases). In nine patients, the grade of resection was total or subtotal. In one case, it was only possible to perform a biopsy. There were two cases with longer survival (9 and 16 months), and their tumors were resected in total or subtotal manner and received adjuvant therapy (radiotherapy and chemotherapy).
Conclusions
Preliminary results, show that in older children, we can improve their survival with the subtotal or total resection of the tumor and the addition of chemotherapy and radiotherapy.
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References
Agranovich AL, Ang LC, Griebel RW, Kobrinsky NL, Lowry N, Tchang SP (1992) Malignant rhabdoid tumor of the central nervous system with subarachnoid dissemination. Surg Neurol 37:410–414
Ahmad FU, Suri A, Mahapatra AK, Mehta VS, Garg A, Sharma MC, Sridhar E (2005) Intraventricular rhabdoid tumor. Indian J Pediatr 72:693–696
Biegel J, Rorke LB, Packer RJ, Emanuel BS (1990) Monosomy 22 in rhabdoid or atypical tumors of the brain. J Neurosurg 73:710–714
Biggs PJ, Garen PD, Powers JM, Garvin J (1987) Malignant rhabdoid tumor of the central nervous system. Hum Pathol 18:332–337
Caldemeyer KS, Smith RR, Azzarelli B, Boaz JC (1994) Primary central nervous system malignant rhabdoid tumor: CT and MR appearance simulates a primitive neuroectodermal tumor. Pediatr Neurosurg 21:232–236
Cuesta T, León B, Rivas A, Martínez F, Cuevas F, Ortiz C (2001) Tumor rabdoide/teratoide atípico. Presentación de tres casos y revisión de la literatura. Rev Neurol 32:618–624
Hirth A, Pedersen PH, Wester K, Mörk S, Helgestad J (2003) Cerebral atypical/rhabdoid tumor of infancy: long-term survival after multimodal treatment, also including triple intrathecal chemotherapy and gamma knife radiosurgery—case report. Pediatr Hematol Oncol 20:327–332
Martínez-Lage JF, Nieto A, Sola J, Domingo R, Costa TR, Poza M (1997) Primary malignant rhabdoid tumor of the cerebellum. Childs Nerv Syst 13:418–421
Muller M, Hubbard SL, Fukuyama K, Dirks P, Matsuzawa K, Rutka JT (1995) Characterization of a pineal region malignant rhabdoid tumor. Pediatr Neurosurg 22:204–209
Muñoz A, Carrasco A, Muñoz MJ, Esparza J (1995) Cranial rhabdoid tumor with marginal cystic component and extra axial extension. AJNR Am J Neuroradiol 16:1727–1728
Olson TA, Bayar E, Kosnik E, Hamoudi AB, Klopfenstein KJ, Pieters RS, Ruymann FB (1995) Successful treatment of disseminated central nervous system malignant rhabdoid tumor. J Pediatr Hematol Oncol 17:71–77
Rorke LB, Packer RJ, Biegel JA (1996) Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood: definition of an entity. J Neurosurg 85:56–65
Satoh H, Goishi J, Sogabe T, Uozumi T, Kiya K, Migita K (1993) Primary malignant rhabdoid tumor of the central nervous system: case report and review of the literature. Surg Neurol 40:429–434
Tekautz TM, Fuller CE, Blaney S, Fouladi M, Broniscer A, Merchant TE, Krasin M, Dalton J, Hale G, Kun LE, Wallace D, Gilbertson RJ, Gajjar A (2005) 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 23:1491–1499
Zimmerman MA, Goumnerova LC, Proctor M, Scott RM, Marcus K, Pomeroy SL, Turner CD, Chi SN, Chordas C, Kieran MW (2005) Continuous remission of newly diagnosed and relapsed central nervous system atypical teratoid/rhabdoid tumor. J Neurooncol 72:77–84
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de León-Bojorge, B., Rueda-Franco, F. & Anaya-Jara, M. Central nervous system atypical teratoid rhabdoid tumor: experience at the National Institute of Pediatrics, Mexico City. Childs Nerv Syst 24, 307–312 (2008). https://doi.org/10.1007/s00381-007-0464-9
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DOI: https://doi.org/10.1007/s00381-007-0464-9