Table 1 Clinicopathologic features of non-desmoplastic small round cell tumors with EWSR1-WT1 Fusions.
Case | Study | Age, sex | Location | Morphology | Keratins | Desmin | SMA/h-caldesmon | Gene fusion | Metastases | Therapy | Patient outcome |
|---|---|---|---|---|---|---|---|---|---|---|---|
1 | Present series | 46, F | Uterine cervix | Bland ovoid to spindled cells, hyalinized vessels, pseudovascular spaces, absent desmoplasia | Positive (rare cells) | Positive | Positive/negative | EWSR1 exon 9-WT1 exon 8 | None | Resection | ANED, 9 months |
2 | Present series | 30, F | Multifocal (uterus, ovaries, retroperitoneum, peritoneum, pelvis) | Bland ovoid to spindled cells, hyalinized vessels, pseudovascular spaces, absent desmoplasia | Positive | Positive | Positive/negative | EWSR1 exon 9-WT1 exon 8 | Extensive intra-abdominal disease at presentation | Resection | AWD, 2 small abdominopelvic lymph node metastases developed after resection, considering stereotactic body radiotherapy, 7 months |
3 | Present series | 36, F | Vagina | Monomorphic spindle cell sarcoma, absent desmoplasia | Positive (rare cells) | Positive | Positive/negative | EWSR1 exon 10-WT1 exon 7 | None | Neoadjuvant chemo-radiotherapy | ANED, 3.5 years |
4 | Ud Din et al [14] | 34, M | Cauda equina | Glomoid cells, myxoid and hyalinized stroma, rosette-like structures | Negative | Positive | Positive/NP | EWSR1 exon 7-WT1 exon 8 | None | Partial resection, eventual radiotherapy and tumor embolization | AWD, 10 years and 9 months |
5 | Alaggio et al [13] | 11, M | Pelvis | Spindled to focally epithelioid tumor resembling leiomyosarcoma, absent desmoplasia | Positive | Positive | Positive/rare cells | EWSR1-WT1 | None | Resection | ANED, 40 months |
6 | Alaggio et al [13] | 9, M | Small intestine | Spindled to focally epithelioid tumor resembling leiomyosarcoma, absent desmoplasia | Positive | Positive | Positive/ rare cells | EWSR1-WT1 | Liver, lymph nodes, omentum | Resection of metastases, multiagent chemotherapy | ANED, 13 years |
