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
Natural History
If left untreated, patients with atypical teratoid rhabdoid tumor may progress to develop headache, hemiparesis, leptomeningeal dissemination, and ultimately death.[1]
Complications
Metastatic spread is noted in approximately one-third of the AT/RT cases at the time of diagnosis and tumors can occur anywhere throughout the CNS. The ASCO study of the 188 documented AT/RT cases prior to 2004 found 30% of the cases had metastasis at diagnosis. Metastatic spread to the meninges (leptomenigeal spread sometimes referred to as sugar coating) is common both initially and with relapse. Average survival times decline with the presence of metastasis. Primary CNS tumors metastasize only within the CNS.
One case of metastatic disease to the abdomen via ventriculoperitoneal shunt has been reported with AT/RT . Metastatic dissemination via this mechanism has been reported with other brain tumors including germinomas, medulloblastomas, astrocytomas, glioblastomas, ependymomas and endodermal sinus tumors. Guler and Sugita separately reported cases of lung metastasis without a shunt.[2][3]
Prognosis
- Prognosis is generally poor, and the 5-year overall survival rate of patients with atypical teratoid rhabdoid tumor is 29 ± 9%.[4]
- 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 prognostifc factor for atypical teratoid rhabdoid tumor.
- Poor prognostic factors for atypical teratoid rhabdoid tumor include:[5]
- Germline mutation
- Age < 2 years
- Metastases at diagnosis
- Subtotal resection
- Large tumors
- Recurrence
References
- ↑ 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.
- ↑ Güler E, Varan A, Söylemezoglu F; et al. (2001). "Extraneural metastasis in a child with atypical teratoid rhabdoid tumor of the central nervous system" (PDF). J. Neurooncol. 54 (1): 53–6. PMID 11763423.
- ↑ Sugita Y, Takahashi Y, Hayashi I, Morimatsu M, Okamoto K, Shigemori M (1999). "Pineal malignant rhabdoid tumor with chondroid formation in an adult". Pathol. Int. 49 (12): 1114–8. PMID 10632935.
- ↑ 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