| Atypical teratoid rhabdoid tumor | |
|---|---|
 | |
| MRI of an AT/RT | |
| Specialty | Neuro-oncology |
| Usual onset | Age 3 and younger[1] |
| Prognosis | Five-year survival rate: 32.2%[2] |
| Frequency | ~58 new diagnoses per year (United States)[2] |
An atypical teratoid rhabdoid tumor (AT/RT) is a rare tumor usually diagnosed in childhood. Although usually a brain tumor, AT/RT can occur anywhere in the central nervous system (CNS), including the spinal cord. About 60% will be in the posterior cranial fossa (particularly the cerebellum). One review estimated 52% in the posterior fossa, 39% are supratentorial primitive neuroectodermal tumors (sPNET), 5% are in the pineal, 2% are spinal, and 2% are multifocal.[3]
In the United States, three children per 1,000,000 or around 30 new AT/RT cases are diagnosed each year. AT/RT represents around 3% of pediatric cancers of the CNS.[4] Around 17% of all pediatric cancers involve the CNS, making these cancers the most common childhood solid tumor.[citation needed] The survival rate for CNS tumors is around 60%. Pediatric brain cancer is the second-leading cause of childhood cancer death, just after leukemia. Recent trends suggest that the rate of overall CNS tumor diagnosis is increasing by about 2.7% per year. As diagnostic techniques using genetic markers improve and are used more often, the proportion of AT/RT diagnoses is expected to increase.
AT/RT was only recognized as an entity in 1996 and added to the World Health Organization Brain Tumor Classification in 2000 (Grade IV).[5] The relatively recent classification and rarity has contributed to initial misdiagnosis and nonoptimal therapy. This has led to a historically poor prognosis.[6]
Current research is focusing on using chemotherapy protocols that are effective against rhabdomyosarcoma in combination with surgery and radiation therapy.
Recent studies using multimodal therapy have shown significantly improved survival data. In 2008, the Dana-Farber Cancer Institute in Boston reported two-year overall survival of 53% and event-free survival of 70% (median age at diagnosis of 26 months).[7] In 2013, the Medical University of Vienna reported five-year overall survival of 100%, and event-free survival of 89% (median age at diagnosis of 24 months).[8]
Survival rates can be significantly improved when the correct genetic diagnosis is made at the outset, followed with specific multimodal treatment.
- ^ "Atypical Teratoid Rhabdoid Tumor (ATRT)". St. Jude Children's Research Hospital. Retrieved Mar 8, 2023.
- ^ a b "Atypical Teratoid Rhabdoid Tumor (ATRT) Diagnosis and Treatment". National Cancer Institute. Retrieved Mar 8, 2023.
- ^ Kieran MW (2006). "An Update on Germ Cell Tumors, Atypical Teratoid/Rhaboid Tumors, and Choroid Plexus Tumors Rare Tumors 3: Brain Tumors---Germ Cell Tumors, Atypical Teratoid/Rhabdoid Tumors, and Choroid Plexus Tumors". American Society of Clinical Oncology. Education. Book. Archived from the original on 2008-01-07. Retrieved 2007-05-20.
- ^ Measure D6: Types of Childhood Cancer – 2006 Tables D6a & D6b. U.S. Environmental Protection Agency. Retrieved on 2008-04-17.
- ^ Kleihues P (2000). Pathology and genetics of tumours of the nervous system. Lyon: IARC Press. ISBN 92-83-22409-4.
- ^ Tekautz TM, Fuller CE, Blaney S, Fouladi M, Broniscer A, Merchant TE, et al. (March 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". Journal of Clinical Oncology. 23 (7): 1491–1499. doi:10.1200/JCO.2005.05.187. PMID 15735125. See Figure 1.
- ^ Chi SN, Zimmerman MA, Yao X, Cohen KJ, Burger P, Biegel JA, et al. (January 2009). "Intensive multimodality treatment for children with newly diagnosed CNS atypical teratoid rhabdoid tumor". Journal of Clinical Oncology. 27 (3): 385–389. doi:10.1200/JCO.2008.18.7724. PMC 2645855. PMID 19064966.
- ^ Slavc I, Chocholous M, Leiss U, Haberler C, Peyrl A, Azizi AA, et al. (February 2014). "Atypical teratoid rhabdoid tumor: improved long-term survival with an intensive multimodal therapy and delayed radiotherapy. The Medical University of Vienna Experience 1992-2012". Cancer Medicine. 3 (1): 91–100. doi:10.1002/cam4.161. PMC 3930393. PMID 24402832.