Table 1.
Clinical Characteristics of Patients With CNS NB-FOXR2 and ETMR and Available Clinical Data
| CNS NB-FOXR2 (n = 63) | ETMR (n = 52) | |||
|---|---|---|---|---|
| No. | (%) | No. | (%) | |
| Age at diagnosis | ||||
| <3 years | 11 | (17) | 34 | (66) |
| ≥3 years | 52 | (83) | 18 | (34) |
| Sex | ||||
| Female | 36 | (57) | 29 | (56) |
| Male | 27 | (43) | 23 | (44) |
| Location of primary tumor | ||||
| Supratentorial | 62 | (98) | 44 | (84) |
| Infratentorial | 5 | (10) | ||
| Supra- and infratentorial | 3 | (6) | ||
| Not known | 1 | (2) | ||
| Staging at diagnosis | ||||
| Localized with complete resection (M0R0)a | 23 | (37) | 20 | (38) |
| Localized with incomplete resection (M0R+)a | 27 | (41) | 17 | (33) |
| Second surgery within primary treatment | 6/27 | 9/17 | ||
| GTR at second surgery | 5/6 | 1/9 | ||
| Localized resection status unknown (M0Rx)a | 2 | (3) | 1 | (2) |
| Microscopic spread to CSF only (M1) | 0 | 3 | (6) | |
| Intracranial/spinal leptomeningeal metastases (M2/3) | 10 | (16) | 10 | (19) |
| Not known | 1 | (3) | 1 | (2) |
| Treatment | ||||
| Within or according to a CNS-ET trial | 53 | (84) | 43 | (83) |
| Individual treatment | 9 | (14) | 6 | (11) |
| Treatment based on protocol for other entityb | 1 | (2) | 0 | |
| No antitumor treatment (early death after surgery) | 0 | 3 | (6) |
Abbreviations: CNS-ET, CNS embryonal tumor; CSF, cerebrospinal fluid; ETMR, embryonal tumor with multilayered rosettes; GTR, gross total resection; M0, localized disease, no metastases; M1, microscopic metastases to CSF; M2/3 intracranial or spinal leptomeningeal metastases visible on MRI, according to the Chang-classification.37
aInformation on CSF staging is missing for n = 6 ETMR patients with localized disease.
bn = 1 patient with CNS NB-FOXR2 was treated according to an ependymoma protocol.