Soft tissue tumor.ppt
- 1. Soft Tissue Tumors Yi-Che,William, Chang Chien, MD, PhD
- 2. Soft Tissue Tumors Lecture Goals: 1. Nomenclature & classification system of tumors 2. Differences between benign and malignant tumors 3. Clinical & morphologic features of common tumors 4. Approach to diagnosis of soft tissue tumors
- 4. • Rare: 50/million, 1% of all cancers (in the US) • >257 subtypes by WHO classification • Can arise virtually anywhere • skin, subcutis, deep soft tissue • organs (i.e. non soft tissue sites) • Seen in general surgical pathology practice • Can mimic • carcinoma • lymphoma • melanoma Epidemiology of Soft Tissue Tumor:
- 5. Soft Tissue Tumor Neoplastic conditions arising in extraskeletal mesodermal tissues -- Skeletal muscle -- Fibrous tissue -- Smooth muscle -- Blood & lymphatic vessels -- Peripheral nerves - neuroectoderm -- Adipose tissue
- 6. Soft Tissue Tumor General principles for diagnosis (Not Always!!): -- Deep lesions tend to be malignant -- Larger tumors tend to be malignant Location Size -- Superficial lesions - benign -- Rapidly growing - malignant Growth pattern -- Infiltrating - malignant Metastasis -- Malignant
- 7. Soft Tissue Tumors Histological morphology Immunohistochemistry Molecular analysis Ultrastructure (rarely used nowadays) Approach to Diagnosis
- 8. Soft Tissue Tumors Cytokeratin (ca.?) Vimentin (not ca.?) Smooth muscle actin Desmin (myogenic?) S-100 (neurogenic?) CD 31 (vasculogenic ?) CD 34 (whatever…) Immunohistochemistry
- 9. Soft Tissue Tumors t( 2:13) Alveolar rhabdomyosarcoma t( 11;22) Ewing’s sarcoma/PNET t( 11;22) Desmoplastic small round cell tumor t( 12;16) Myxoid liposarcoma t( 9;22) Myxoid chondrosarcoma t(12;22) Clear cell sarcoma t( X;18) Synovial sarcoma Cytogenetic Changes
- 11. FNCLCC Grading System Fe´de´ration Nationale des Centres de Lutte le Cancer Jean-Michel Coindre: Grading of Soft Tissue Sarcomas; Review and Update, Arch Pathol Lab Med. 2006;130:1448–1453
- 12. Staging: American Joint Committee on Cancer (AJCC ) of soft tissue sarcomas classification(8 ed.)
- 13. Nodular Fasciitis Probably secondary to trauma On forearm, trunk, back- well circumscribed Spindle cells ( fibroblasts & myofibroblasts) in loose matrix -“fibroblasts in tissue culture” Rapid growth, frequent mitotic figures Self-limited, cured by excision Must differentiate from a sarcoma USP6 gene translocation identified (neoplastic).
- 15. Fibromatosis Fibroproliferative lesions Desmoid - Infiltrative masses in abdominal, extra-abdominal & intra-abdominal Fibroblasts & Myofibroblasts Palmar, plantar fibromatosis Surgical excision Beta-catenin/ AFP gene mutation Infiltrative growth pattern Tendency to recur
- 16. Fibromatosis
- 17. Fibrosarcoma Malignant tumor of fibroblast origin In adults, in lower extremities, upper extremities, trunk Increased cellularity, high nuclear-cytoplasmic ratios Spindle cells in a herringbone pattern Must exclude other tumors which resemble fibrosarcoma - Peripheral nerve sheath tumor - Synovial sarcoma
- 18. Fibrosarcoma
- 19. Dermatofibroma Benign fibrous histiocytoma Usually in skin Mixture of fibroblasts, myofibroblasts, histiocytes Surgical excision
- 20. Dermatofibroma
- 21. Dermatofibrosarcoma Protuberans (DFSP) Fibrohistiocytic tumor Intermediate malignant potential In skin & subcutis Spindle cells in storiform pattern Local recurrence CD 34 Transformation to fibrosarcoma Surgical excision
- 22. Dermatofibrosarcoma Protuberans ( DFSP )
- 23. Undifferentiated Pleomorphic Sarcoma (MFH) Malignant soft tissue tumor without specific lineage differentiation Rare type of soft-tissue sarcoma Most frequently encountered sarcoma post radiation therapy In adults, in deep soft tissue - extremities & retroperitoneum Marked pleomorphism, spindle cells, storiform, myxoid
- 24. MFH
- 25. Leiomyoma Benign soft tissue tumor Arising in subcutaneous tissue or blood vessel wall Usually painful Fascicles of regular smooth muscle cells
- 26. Leiomyoma
- 27. Leiomyosarcoma Malignant soft tissue tumor Arising in extremities of blood vessel wall Necrosis, hemorrhage Fascicles, nuclear atypia High mitotic activity
- 28. Leiomyosarcoma
- 29. Rhabdomyosarcoma Malignant tumor of striated muscle differentiation In children & young adults Several subtype - Embryonal (Botryoid) - Alveolar - Pleomorphic -Spindle/Sclerotic -Epithelioid
- 30. Embryonal Rhabdomyosarcoma Most common in children, in head & neck Most common subtype Rhabdomyoblasts with cytoplasmic cross-striation
- 32. Alveolar Rhabdomyosarcoma In extremities Fibrous septa with loose clusters of rounded cells in center - alveolar pattern
- 34. Lipoma Benign, well-circumscribed tumor of well-differentiated adipocytes Usually subcutaneous, any site of adipose tissue Most common type of benign soft tissue tumor In adult, upper back, neck, shoulder Resemble normal adipose tissue Subtypes:angiolipoma, spindle cell lipoma
- 35. Lipoma
- 36. Liposarcoma Second most common sarcoma in adults In deep compartments of extremities & retroperitoneum Lipoblasts Several subtypes - Well differentiated/atypical lipomatous tumor -Dedifferentiated - Myxoid - Pleomorphic
- 37. Liposarcoma
- 38. Hemangioma Benign lesion Resemble normal blood vessels Congenital or non-congenital Most common in infants & children Head & neck, internal organs - liver
- 40. Angiosarcoma Malignant vascular tumor Many locations: skin, soft tissue, breast, live, spleen Irregular channels & atypical endothelial cells CD31, CD34, ERG Fli1+
- 41. Angiosarcoma
- 43. Schwannoma Benign tumor of neural differentiation - Schwann cells Antoni A Antoni B In association with large nerve trunks Head & neck, extremities -- Cellular area, palisaded nuclei -- Less cellular area, myxoid background S-100
- 44. Schwannoma
- 46. Synovial sarcoma Malignant soft tissue tumor Arising in region of a joint & other sites of deep soft tissue Unknown origin- misnomer !! Multilobular Biphasic pattern - spindle cells - epithelial - like cells t( X;18 )
- 47. Synovial sarcoma