TABLE 1.
Clinical data for the 20 patients with intracranial mesenchymal tumors with FET‐CREB fusions
| Patient ID | Age | Sex | Fusion | Tumor location | Extent of resection | Initial adjuvant therapy | Disease progression/recurrence | Clinical status at last follow up | Length of follow‐up |
|---|---|---|---|---|---|---|---|---|---|
| ATF1 #1 | 12 | M | EWSR1‐ATF1 | Cerebral convexity (parietal) | Subtotal | XRT (59.4 Gy) | Local recurrence x 2 (6 months; 10 months) | Alive with progressive disease | 24 months |
| ATF1 #2 | 9 | F | EWSR1‐ATF1 | Cerebral convexity (frontal) | Subtotal | None | Local recurrence (3 months) | Died of disease | 63 months |
| ATF1 #3 | 24 | F | EWSR1‐ATF1 | Cerebral convexity (occipital) | Unknown | Unknown | Unknown | Unknown | Unknown |
| ATF1 #4 | 13 | F | EWSR1‐ATF1 | Cerebral convexity (frontal) | Gross total | None | None | Alive, disease‐free | 24 months |
| ATF1 #5 | 34 | F | EWSR1‐ATF1 | Tentorium | Subtotal | None | Local recurrence (54 months) | Alive, disease‐free | 81 months |
| ATF1 #6 | 17 | F | EWSR1‐ATF1 | CP angle | Subtotal | XRT (59.4 Gy); ifosfamide, vincristine, adriamycin | Metastasis to thoracic lymph nodes and vertebrae (12 months) | Died of disease | 27 months |
| ATF1 #7 | 70 | M | EWSR1‐ATF1 | CP angle with spinal dissemination | Subtotal | None | Continuous progression | Died of disease | 1 month |
| ATF1 #8 | 17 | F | EWSR1‐ATF1 | CP angle | Subtotal | None | None | Alive with stable disease | 13 months |
| CREB1 #1 | 14 | F | EWSR1‐CREB1 | Lateral ventricle | Gross total | None | None | Alive, disease‐free | 59 months |
| CREB1 #2 | 39 | F | EWSR1‐CREB1 | Lateral ventricle | Gross total | None | None | Alive, disease‐free | 6 months |
| CREB1 #3 | 10 | M | EWSR1‐CREB1 | Falx (parietal) | Gross total | None | Local recurrence (9 months) | Alive, disease‐free | 57 months |
| CREB1 #4 | 14 | F | EWSR1‐CREB1 | Lateral ventricle | Unknown | None | None | Alive, disease‐free | 47 months |
| CREB1 #5 | 25 | F | EWSR1‐CREB1 | CP angle | Gross total | None | Local recurrence (11 months) | Alive with stable disease | 30 months |
| CREB1 #6 | 14 | F | EWSR1‐CREB1 | Cerebral convexity (parietal) | Unknown | None | Local recurrence (49 months) | Alive with progressive disease | 57 months |
| CREB1 #7 | 12 | M | EWSR1‐CREB1 | Tentorium | Gross total | None | None | Alive, disease‐free | 46 months |
| CREM #1 | 15 | F | EWSR1‐CREM | Spinal cord (thoracic) | Subtotal | XRT (dose unknown) | Local recurrence (6 months) | Alive, disease‐free | 30 months |
| CREM #2 | 14 | F | EWSR1‐CREM | Lateral ventricle | Gross total | None | None | Alive, disease‐free | 38 months |
| CREM #3 | 5 | F | EWSR1‐CREM | Cerebral convexity (frontal) | Subtotal | ifosfamide, carboplatin, etoposide | Local recurrence x 2 (2 months; 6 months) | Alive with progressive disease | 11 months |
| CREM #4 | 30 | M | EWSR1‐CREM | Falx (frontal) | Subtotal | XRT (54 Gy) | None | Alive, disease‐free | 6 months |
| FUS #1 | 4 | F | FUS‐CREM | Cerebral convexity (occipital) | Gross total | None | Local recurrence x 2 (9 months; 13 months) | Alive, disease‐free | 36 months |
Patients CREB1 #4 and CREB1 #7 have been previously described in part as case 2 and case 1 from Bale et al Brain Pathology 2018.