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Soft tissue tumours
- Soft tissue tumors arise from non-epithelial connective tissues like fat, muscle, fibrous tissues, blood vessels, or deep skin tissues. They represent less than 1% of cancers. - Soft tissue tumors are classified based on the tissue of origin and characteristics like morphology, immunohistochemistry, and genetics. Major categories include adipose, fibrous, skeletal muscle, smooth muscle, and tumors of uncertain origin. - Specific tumor types discussed in detail include lipoma, liposarcoma, nodular fasciitis, fibromatosis, rhabdomyosarcoma, leiomyoma, leiomyosarcoma, synovial sarcoma, and undifferent
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Soft tissue tumours
- 1. SOFT TISSUE TUMORS BY:DR. USMAN SHAMS
- 2. INTRODUCTION • Soft tissuerefers to non-epithelial tissue excluding the skeleton, joints, central nervous system, hematopoietic and lymphoid tissues • The area of soft tissue pathology is restricted to neoplasms. • Less than 1% of all cancers • 2% of all cancer mortality
- 3. PATHOGENESIS • Sporadic withno known predisposing cause. • Germline mutations in tumor suppressor genes • Neurofibromatosis 1 • Gardner syndrome • Li-Fraumeni syndrome • Osler-Weber Rendu syndrome • Known environmental exposures such as radiation, burns or toxins. • No known precursor lesion
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- 5. CLASSIFICATION • Classification ofsoft tissue tumors continues to evolve as new molecular genetic abnormalities are identified. • Pathologic classification integrates morphology (e.g., muscle differentiation), immunohistochemistry and molecular diagnostics
- 7. ADIPOSE TISSUE • Lipoma •Liposarcoma • Well-differentiated • Myxoid • Pleomorphic
- 8. LIPOMA • Benign tumorof fat • Most common soft tissue tumor of adulthood. • Conventional lipoma, fibrolipoma, angiolipoma, spindle cell lipoma and myelolipoma. • Soft, mobile, and painless (except angiolipoma) • Usually cured by simple excision.
- 12. LIPOSARCOMA • One ofthe most common sarcomas of adulthood • People in their 50s to 60s • Deep soft tissues of the extremities and in the retroperitoneum • Amplification of MDM2 gene
- 13. • Well-differentiated liposarcoma •Adipocytes with scattered atypical spindle cells • Myxoid liposarcoma • Abundant basophilic extracellular matrix, arborizing capillaries and primitive cells at various stages of adipocyte differentiation • Pleomorphic liposarcoma • Sheets of anaplastic cells, bizarre nuclei and variable amounts of immature adipocytes (lipoblasts).
- 20. FIBROUS TUMORS • Nodularfasciitis • Fibromatosis
- 21. NODULAR FASCIITIS • Self-limited •Fibroblastic and myofibroblastic proliferation • Young adults • Upper extremity • History of trauma • Rapid growth over a period of several weeks or months • Typically no larger than 5 cm • Spontaneously regresses and if excised, it rarely recurs.
- 22. • Circumscribed, orslightly infiltrative • Arranged randomly or in short fascicles reminiscent of tissue culture fibroblasts • The cells vary in size and shape (spindle to stellate) and have conspicuous nucleoli; mitotic figures are abundant. • Lymphocytes and extravasated red blood cells are common.
- 26. FIBROMATOSES • Superficial Fibromatosis •An infiltrative fibroblastic proliferation that can cause local deformity but has an innocuous clinical course • Palmar (Dupuytren contracture) • Plantar • Penile (Peyronie disease) • Deep Fibromatosis (Desmoid Tumors) • Large, infiltrative masses that frequently recur but do not metastasize • Mutations in the APC or -catenin genesβ
- 27. Palmar Fibromatosis (Dupuytrencontracture)
- 28. • Gray-white, firm,poorly demarcated masses • Varying from 1 to 15 cm in greatest diameter • Rubbery and tough • Marked infiltration of surrounding muscle, nerve and fat. • Cytologically bland fibroblasts arranged in broad sweeping fascicles amid dense collagen
- 30. SKELETAL MUSCLE TUMORS •Rhabdomyoma • Rhabdomyosarcoma
- 31. RHABDOMYOSARCOMA • Malignant mesenchymaltumor with skeletal muscle differentiation • Most common soft tissue sarcoma of childhood and adolescence • Head and neck and genitourinary tract
- 32. • Embryonal (60%) Primitivespindle cells, “strap cells” • Alveolar (20%) Uniform round discohesive cells between septa • Pleomorphic (20%)
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- 38. SMOOTH MUSCLE TUMORS •Leiomyoma • Leiomyosarcoma
- 39. LEIOMYOMA • Uterine Leiomyoma •Pilar Leiomyoma (Erector pili muscle in skin) • Hereditary leiomyomatosis and renal cell cancer syndrome
- 42. LEIOMYOSARCOMA • 10% to20% of soft tissue sarcomas • Adults • Women more frequently than men • Deep soft tissues of the extremities and retroperitoneum • Great vessels, especially the inferior vena cava • Painless firm masses • Retroperitoneal tumors - abdominal symptoms
- 46. SMOOTH MUSCLE ACTIN(SMA)
- 47. TUMORS OF UNCERTAINORIGIN • Synovial sarcoma • Undifferentiated pleomorphic sarcoma
- 48. SYNOVIAL SARCOMA • Misnomer •10% of all soft tissue sarcomas • 20s to 40s age • Deep-seated mass • t(x;18)(p11;q11) • Monophasic synovial sarcoma • Uniform spindle cells with scant cytoplasm and dense chromatin growing in short, tightly packed fascicles • Biphasic synovial sarcoma • Spindle cell component + gland-like structures • Epithelial markers (e.g., keratins)
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