Table 1.
Published reviews and clinical trials that included or were specific to atypical teratoid rhabdoid tumors.
| Study time period | n of patients | Age | Type of study | Chemotherapy | Radiation | Survival | ||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Tekautz et al. (2005) | 31 | 22 patients <3 years (median 1 year) 9 patients ≥3 years (median 3.9 years) |
Retrospective review | Multiple regimens | <3 years 2 local, 1 CSI + boost | <3 years (estimates) 2-year EFS 11 ± 6% 2-year OS 17 ± 8% |
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| July 1984–June 2003 | ≥3 years 7 patients CSI + boost | ≥3 years (estimates) 2-year EFS 78 ± 14% 2-year OS 89 ± 11% |
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| Geyer et al. (2005) | 28 | 12 patients aged 0–11 months. | Phase II/III | Induction A (n = 16) VCR/CDDP/CTX/VP | 2 patients prior to progression (1 focal, 1 CSI) | 1-year EFS 32 ± 9% 2-year EFS 14 ± 7% | ||||||
| CCG9921 | 6 patients aged 12–17 months. | Induction B (n = 12) VCR/CDDP/IFOS/VP | 5-year EFS 14 ± 7% 5-year OS 29 ± 9% | |||||||||
| April 1993–June 1997 | 10 patients aged 18–36 months. | Maintenance VCR/CDDP/CTX/VP | ||||||||||
| Douglas Strother [personal communication 2011] POG9233/34 1992–1998 |
33 | All patients <3 years | Phase III | CTX/VCR/CDDP/VP Standard versus dose-intensified | None | 5 year OS 0% Median survival 6.7 months. | ||||||
| Lafay-Cousin et al. (2012) | 50 | Median age 16.7 months. | Retrospective review | Multiple regimens | 21 patients at some point during therapy | 2-year OS 36.4 ± 7.7% | ||||||
| 17 patients aged <12 months. | 22 conventional | |||||||||||
| 21 patients aged 12–36 months. | 18 high-dose chemo | 6 patients at time of relapse | ||||||||||
| 1995–2007 | 12 patients >36 months. | |||||||||||
| Chi et al. (2009) | 20 | Median 26 months (2.4 month–9.5 years) | Phase II | Modified IRS-III | 54 Gy focal (n = 11) | 2-year PFS 53 ± 13% | ||||||
| 36 Gy CSI + boost (n = 4) | 2-year OS 70± 10% | |||||||||||
| 2004–2006 |
CDDP, cisplatin; CSI, craniospinal radiation; CTX, cyclophosphamide; EFS, event free survival; IFOS, ifosfamide; IRS, Intergroup rhabdomyosarcoma study; OS, overall survival; PFS, progress free survival; VCR, vincristine; VP, etoposide.