Atypical teratoid rhabdoid tumor natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]
Overview
If left untreated, patients with atypical teratoid rhabdoid tumor may progress to develop obstructive hydrocephalus, leptomeningeal dissemination, and ultimately death.[1] Common complications of atypical teratoid rhabdoid tumor include obstructive hydrocephalus, leptomeningeal dissemination, and recurrence.[1] Prognosis is generally poor, and the 5-year overall survival rate of patients with atypical teratoid rhabdoid tumor is 29 ± 9%.[2]
Natural History
- If left untreated, patients with atypical teratoid rhabdoid tumor may progress to develop obstructive hydrocephalus, leptomeningeal dissemination, and ultimately death.[1]
Complications
- Obstructive hydrocephalus
- Leptomeningeal dissemination
- Intratumoral hemorrhage
- Recurrence
Prognosis
- Prognosis is generally poor, and the 5-year overall survival rate of patients with atypical teratoid rhabdoid tumor is 29 ± 9%.[2]
- The patients usually succumb to atypical teratoid rhabdoid tumor between 6 months and 1 year from diagnosis.
- Compared to medulloblastoma, atypical teratoid rhabdoid tumor has a significantly worse prognosis.
- Older age at diagnosis is a favorable prognostic factor for atypical teratoid rhabdoid tumor.
- Poor prognostic factors for atypical teratoid rhabdoid tumor include:[4]
- Germline mutation
- Age < 2 years
- Metastases at diagnosis
- Subtotal resection
- Large tumors
- Recurrence
References
- ↑ 1.0 1.1 1.2 1.3 Chan KH, Mohammed Haspani MS, Tan YC, Kassim F (2011). "A case report of atypical teratoid/rhabdoid tumour in a 9-year-old girl". Malays J Med Sci. 18 (3): 82–6. PMC 3216225. PMID 22135607.
- ↑ 2.0 2.1 Ginn, Kevin F.; Gajjar, Amar (2012). "Atypical Teratoid Rhabdoid Tumor: Current Therapy and Future Directions". Frontiers in Oncology. 2. doi:10.3389/fonc.2012.00114. ISSN 2234-943X.
- ↑ Udaka, Y. T.; Shayan, K.; Chuang, N. A.; Crawford, J. R. (2013). "Atypical Presentation of Atypical Teratoid Rhabdoid Tumor in a Child". Case Reports in Oncological Medicine. 2013: 1–4. doi:10.1155/2013/815923. ISSN 2090-6706.
- ↑ Prognosis of atypical teratoid rhabdoid tumor. National Cancer Institute 2015. http://www.cancer.gov/types/brain/hp/child-cns-atrt-treatment-pdq. Accessed on December 14, 2015