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. Common complications of atypical teratoid rhabdoid tumor include obstructive hydrocephalus, leptomeningeal dissemination, and recurrence. Prognosis is generally poor, and the 5-year overall survival rate of patients with atypical teratoid rhabdoid tumor is 29 ± 9%.
Natural History, Complications, and Prognosis
Natural History
- If left untreated, patients with atypical teratoid rhabdoid tumor may progress to develop obstructive hydrocephalus, leptomeningeal dissemination, and ultimately death.[1]
Complications
- Common complications of atypical teratoid rhabdoid tumor include:[1][2]
- 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%.[3]
- 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 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.
- ↑ 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.
- ↑ 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.
- ↑ 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