SOFT TISSUE TUMORS Pleomorphic – aggressive and metastatic
Pathogenesis of Sarcomas FIBROUS TUMORS
- MC sporadic with no known predisposing Nodular Fasciitis cause; some are linked to germline o Self-limited fibroblastic and myofibroblastic mutations in TSGs or environmental proliferation in young adults exposures o MC on the forearm, chest, back - Undefined precursors o Grow rapidly (weeks-months) but regresses - Karyotypic complexity spontaneously; if excised rarely recurs a. Simple (20%) – young patients, o Clonal proliferation harboring a t(17;22) monomorphic; Ewing and synovial translocation that produces a MYH9-USP6 sarcoma fusion b. Complex (80%) – leiomyosarcomas, o Morphology undifferentiated pleomorphic sarcoma Arises in the deep dermis, subcutis, fascia, TUMORS OF ADIPOSE TISSUE or muscle Lipoma Non-encapsulated – well-circumscribed or o MC soft tissue tumor in adults slightly infiltrative, < 3 cm o Morphology (conventional) Plump immature-appearing fibroblasts an Well-encapsulated mass of mature myofibroblasts containing elongated adipocytes nuclei with punctate nucleoli Arise in the subcutis of the proximal extremities and trunk during middle adulthood Lipomatosis – multiple multifocal lipomas in a limb MC soft, mobile, painless, and cured with simple excision Liposarcoma o MC sarcomas of adulthood o Develop in deep soft tissues of the proximal extremities and retroperitoneum in the 6 th-7th decade o Genetic aberrations Well-differentiated: 12q13-q15 (includes MDM2) Myxoid – 1(12;16) translocation o Morphology Well-differentiated – mature adipocytes with scattered atypical spindle cells Myxoid – abundant basophilic ECM, arborizing capillaries, and primitive cells at various differentiation stages (resemble fetal fat) Pleomorphic – sheets of anaplastic cells with bizarre nuclei admixed with variable numbers of immature adipocytes (lipoblasts) o Clinical Features Recur locally unless adequately excised Well-differentiated – indolent