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. Author manuscript; available in PMC: 2021 May 14.
Published in final edited form as: Semin Diagn Pathol. 2020 Jun 5;38(3):222–231. doi: 10.1053/j.semdp.2020.05.005

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.