Incidence
Clinical Behavior
Presentation
Prognosis

Incidence

The exact incidence of childhood central nervous system atypical teratoid/rhabdoid tumor (AT/RT) is difficult to determine since the tumor has been widely recognized only for the last decade. In the Children’s Cancer Group study CCG-9921, 10% of infants had AT/RT.[1] In Pediatric Oncology Group study CCG-9233, 36 (11%) of 319 patients were identified in central review as having AT/RT. A Taiwanese study suggests an incidence of 26% in children aged younger than 3 years.[2] The incidence in older patients is unknown. In The AT/RT Registry, 12 of 42 patients (29%) were older than 36 months at the time of diagnosis.[3]

Childhood AT/RT is a clinically aggressive tumor that primarily occurs in children younger than 3 years but it also can occur in older children and has been reported in adults.[4,5] In about half of patients, the tumor is located in the posterior fossa, although it can occur anywhere in the central nervous system.[1]

Signs and symptoms related to the tumor are dependent on location. Young patients with posterior fossa tumors usually present with symptoms related to hydrocephalus such as early morning headaches, vomiting, and lethargy. They may also develop ataxia or regression of motor skills. Because AT/RT grows rapidly, patients typically have a fairly short history of progressive symptoms measured in days to weeks. Data from The AT/RT Registry suggests that approximately 20% of patients present with disseminated disease.[3] Dissemination is typically through leptomeningeal pathways seeding the spine and other areas of the brain. There are also reports of rare patients with synchronous renal rhabdoid and AT/RT.[6]

Most published information on outcome for patients with AT/RT is based on small series and is retrospective in nature. Initial retrospective studies reported an average survival from diagnosis of only about 12 months.[1,4,7] There are, however, reports of long-term survivors.[8]

References

1. Packer RJ, Biegel JA, Blaney S, et al.: Atypical teratoid/rhabdoid tumor of the central nervous system: report on workshop. J Pediatr Hematol Oncol 24 (5): 337-42, 2002 Jun-Jul.  [PUBMED Abstract]
   
   
2. Ho DM, Hsu CY, Wong TT, et al.: Atypical teratoid/rhabdoid tumor of the central nervous system: a comparative study with primitive neuroectodermal tumor/medulloblastoma. Acta Neuropathol 99 (5): 482-8, 2000.  [PUBMED Abstract]
   
   
3. Hilden JM, Meerbaum S, Burger P, et al.: Central nervous system atypical teratoid/rhabdoid tumor: results of therapy in children enrolled in a registry. J Clin Oncol 22 (14): 2877-84, 2004.  [PUBMED Abstract]
   
   
4. Burger PC, Yu IT, Tihan T, et al.: Atypical teratoid/rhabdoid tumor of the central nervous system: a highly malignant tumor of infancy and childhood frequently mistaken for medulloblastoma: a Pediatric Oncology Group study. Am J Surg Pathol 22 (9): 1083-92, 1998.  [PUBMED Abstract]
   
   
5. Lutterbach J, Liegibel J, Koch D, et al.: Atypical teratoid/rhabdoid tumors in adult patients: case report and review of the literature. J Neurooncol 52 (1): 49-56, 2001.  [PUBMED Abstract]
   
   
6. Biegel JA, Fogelgren B, Wainwright LM, et al.: Germline INI1 mutation in a patient with a central nervous system atypical teratoid tumor and renal rhabdoid tumor. Genes Chromosomes Cancer 28 (1): 31-7, 2000.  [PUBMED Abstract]
   
   
7. Rorke LB, Packer RJ, Biegel JA: Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood: definition of an entity. J Neurosurg 85 (1): 56-65, 1996.  [PUBMED Abstract]
   
   
8. Olson TA, Bayar E, Kosnik E, et al.: Successful treatment of disseminated central nervous system malignant rhabdoid tumor. J Pediatr Hematol Oncol 17 (1): 71-5, 1995.  [PUBMED Abstract]
   
   

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