Table 1.
Overview of mesenchymal neoplasms with SWI/SNF complex deficiency.
Tumor | Age and sex distribution | Anatomic predilection | SWI/SNF subunit genomic inactivation |
---|---|---|---|
Malignant rhabdoid tumor | Infants and children, F = M | Kidney, brain, soft tissue | SMARCB1 |
Epithelioid sarcoma | Mostly adults, M>F | Distal extremities, trunk | SMARCB1 |
Poorly differentiated chordoma | Mostly children, F>M | Skull base/clivus, axial spine | SMARCB1 |
Myoepithelial carcinoma | Children and adults, M>F | Extremities, limb girdle | SMARCB1 |
Extraskeletal myxoid chondrosarcoma | Mostly adults, M>F | Extremities, limb girdle | SMARCB1 |
Epithelioid schwannoma | Mostly adults, F = M | Trunk, extremities | SMARCB1 |
Epithelioid MPNST | Mostly adults, F = M | Trunk, extremities | SMARCB1 |
SMARCA4-deficient thoracic sarcoma | Mostly adults, M>F | Mediastinum, pleura, lung | SMARCA4 |
F: Female, M: Male, MPNST: Malignant peripheral nerve sheath tumor.