Soft tissue neoplasms
Soft
tissue neoplasms
Updated: 08/07/2020
© Jun Wang, MD, PhD
General features
- Non epithelial, extraskeletal tissue, except hematopoietic tissue
- Usually mesodermal in origin, except peripheral nerves (neuroectoderm)
- Classified according to type of cellular differentiation
- Associated with radiation therapy, chemical carcinogens, etc
- 6 large groups
Adipose tissue
Fibrous tissue
Skeletal muscle
Smooth muscle
Blood/lymphatic vessels
Peripheral nervous system
Diagnostic considerations
- History
- Location
- Morphology
- Immunohistochemistry studies
- Cytogenetics/molecular studies
Pathogenesis
- Genetic predisposition: p53 mutation (Li-Fraumeni syndrome; familial adenomatous polyposis and Gardner syndrome, Retinoblastoma, Neurofibromatosis I, Neurofibromatosis II, etc
- Radiation and chemotherapy: Undifferentiated pleomorphic sarcoma, angiosarcoma
- Childhood cancer survivors: Probably associated with radiation and chemotherapy
- Industrial chemicals: Hepatic angiosarcoma, etc
- Chronic edema, chronic irritation, trauma: Lymphangiosarcoma, fibrosarcoma, desmoid tumors, etc
- Viral infections: Kaposi sarcoma
Commonly used markers for soft
tissue tumor
- Desmin: Myogenic differentiation, rhabdomyosarcomas, leiomyoma, leiomyosarcoma, etc
- S100: Neurogenic, adipose and melanocytic differentiation, lipoma, liposarcoma, malignant peripheral nerve sheath tumor, melanoma, etc
- Cytokeratin: Usually epithelial tissue, but negative in soft tissue, except it is positive for synovial sarcoma
- von Willebrand's factor(Factor VIII-related antigen) and CD31: Endothelial differentiation, angiosarcoma, etc
Commonly seen genetic abnormalities
- Atypical lipomatous tumour/well differentiated liposarcoma: Ring or giant marker/rod chromosome
- Myxoid liposarcoma: FUS-DDIT3 (TLS-CHOP: deregulate NF-kB pathway)
- Nodular fasciitis: MYH9-USP6
- Deep fibromatosis: beta-catenin
- Embryonal rhabdomyosarcoma: PAX3
- Alveolar rhabdomyosarcoma: N-myc, PAX3-FKFR, PAX7-FKHR
- Synovial sarcoma: t(X:18):SYT-SSX1, SYT-SSX2
- Malignant peripheral nerve sheath tumor: NF
Some commonly seen soft tissue
tumors
- Adipose tissue tumors
- Atypical lipomatous tumour/well differentiated liposarcoma
- Dedifferentiated liposarcoma
- Myxoid liposarcoma/round cell liposarcoma
- Pleomorphic liposarcoma
- Liposarcoma, not otherwise specified
- Fibrous tumors
- Muscular tumors
- Peripheral nerve tumors
- Vascular tumors and tumor like lesions
- Others
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