Table 4 FET-TFCP2 rhabdomyosarcomas reported in the literature
Author | Case ID | Age (y)/gender | Location | Tumor size | Morphology | IHC | Molecular | Therapy | Survival |
|---|---|---|---|---|---|---|---|---|---|
Dashti et al. [7], 1 case | – | 72/M | Mandible | 4 cm | Monomorphic spindled and epithelioid cells arranged in short fascicles. No stroma. | Des+ MYOD1+ S100 focal+ Keratin+ ALK+ | FUS (exon 6)-TFCP2 (exon 2) | Radical mandibulectomy | 2 Months after surgery disease free |
Zhu et al. [10], 1 case | Case 5 | 74/F | Maxilla | 4 cm | Monomorphic spindle cells with inflammatory background. Frequent mitotic figures and focal necrosis. | Des+ MYOD1+ S100− Keratin− ALK+ | FUS (exon 6)-TFCP2 (exon 2) | NA | Regional metastasis to the neck |
Agaram et al. [8], 4 cases | Case 3 | 27/F | Skull | NA | Epithelioid | Des+ MYOD1+ Keratin + ALK+ | EWSR1-TFCP2 | NA | NA |
Case 4 | 33/F | Maxilla | Epithelioid | EWSR1-TFCP2 | Surgical resection | ANED (108 months FO) | |||
Case 5 | 20/M | Iliac bone | Pure spindle cell morphology | EWSR1-TFCP2 | NA | NA | |||
Case 6 | 37/F | Femoral bone | Pure spindle cell morphology | FUS-TFCP2 | NA | NA | |||
Tagami et al. [9], 1 case | – | 70/F | Lumbar vertebra | NA | Spindled to round cells, scattered rhabdomyoblasts | Des focal + MYOD1 + S100 − Keratin + ALK + | FUS rearrangement | Surgery followed by chemotherapy and radiotherapy | Still alive after 6 month of diagnosis |
Wong et al. [20], 1 case | – | 23/M | Nasal cavity | 8 cm | Monomorphic epithelioid, and rhabdoid and spindled cells. High mitotic count >30 per 10 HPF | Des focal + MYOD1+ MYF4 focal+ S100− Keratin− ALK+ | FUS-TFCP2 | No response to combination of chemotherapy and radiotherapy, currently treated with Crizotinib (ALK inhibitor) | AWD (2 months FO) |
