Table 1.
Feature | Embryonal | Alveolar | Spindle cell/sclerosing | Pleomorphic |
---|---|---|---|---|
Frequent localisation | Mucosae, Urogenital, Head, Neck |
Head and neck, Extremities | Head and neck | Ubiquitous |
Prevalence | 10–20% | 20–30% | 5–10% | 50–60% |
Prognosis | Poor | Poor | Variable | Poor |
Histo-morphology | Round and spindled proliferation within a myxoidstroma | Round cells with fibrous septa | Homogeneous proliferation of spindle-like cells (spindled and sclerosing/epithelioid) | Heterogeneous cells in shape and size (pleomorphism) |
Immunohistochemistry | Heterogeneous MYOD1/myogenin | Diffuse MYOD1/Myogenin | MYOD1 >myogenin | Scant MYOD1/myogenin |
Genetic features | Drivers unknown | FOX01 fusion | VGLL2/NCOA2 fusions, TFCP2 fusions, MYOD1 activatingmutations | Complexgenetics |
RMS, rhabdomyosarcomas.