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This Concept Map, created with IHMC CmapTools, has information related to: Soft tissue tumors, Soft Tissue Tumors Smooth Muscle ????, Soft Tissue Tumors Fibrous Tumors, etc. SUPERFICIAL FIBROMATOSES-MALE>fm (PALMAR, PLANTAR, PENILE FIB'SES) -many are myofibroblasts in fascicles -?trisomies 3, 8 ass'n Palmar-DUPUYTREN CONTRACTURE -thickened palmar fascia -bilateral in 50% -4-5th fingers contracted especially Plantar-similar changes Penile-PEYRONIE DZ-palpable, indurated on DORSOLATERAL aspect ->ABNL curvature, urethral constriction Infantile Digital-hands, ft əyo -1/3 get it at birth, 1/3 have mult tumors -spon regression or recur after excision -can stabilize, can resolve spon'ly, Soft Tissue Tumors Fatty Tumors LIPOMAS -most common adult STT -soft, mobile, painless Can be: Conventional Lipoma -chrom 12 rearrangements -mature adips, encapsulated -SubQ in prox limbs, trunk -mid-adulthood Fibrolipoma Angiolipoma-PAIN Spindle Cell Lipoma Myelolipoma Pleomorphic Lipoma -16q, 13q rearrangements, Soft Tissue Tumors Fibrohistiocytic Tumors MALIGNANT FIBROUS HISTIOCYTOMA -most common MALIGNANT STT in adults -DEEP, PROXIMAL EXTREMITIES -bizarre multinuc cells, STORIFORM -inflamed collagenous stroma, foamy macs -myxoid -some w/ no distinct diff'tion -some variants of liposarcomas, other sarcs MYXOID type-grade proportional to Px -ATYPICAL FIBROXANTHOMAS-cutaneous -rarely disseminate -mostly aggressive, recur often, METS 30-50% ANGIOMATOID TYPE-indolent, in teens, 20s, Soft Tissue Tumors Fibrohistiocytic Tumors DERMATOFIBROSARCOMA PROTUBERANS -young, middle-aged adults -slow, enlarging plaque -trunk, upper limbs -RARE mets, if recurrent->sarcoma MORPH-swiss=cheese, storiform Tx: remove w/ wide margins, Soft Tissue Tumors Fibrous Tumors, etc. MYOSITIS OSSIFICANS -metaplastic bone present -athletic teens, adults -after trauma in 50% -in SubQ, mm. of prox limbs Sx-swollen/painful->firm, painless MORPH-center w/ fibroblasts ->move out to 'blasts->trabec ->eventually ossifies completely ->marrow grows in Tx-removal is curative, Soft Tissue Tumors Fatty Tumors LIPOSARCOMAS -very common-40s-60s -2nd most common MALIGNANT STT -deep prox limbs, retroperitoneum ->LARGE TUMORS Histo: well-diff'd, pleomorphic, round cell, myxoid-both t(12;16) -always have LIPOBLASTS -mimic fetal fat cels Round cell, Pleomorphic-most aggressive -METS frequently-HIGH GRADE -round - t(12;16) Well-diff'd - quite indolent Myxoid-intermediate aggression -MOST COMMON -like fetal fat -t(12;16) All recur if not adequately excised, Soft Tissue Tumors Peripheral Nerve ????, Soft Tissue Tumors Vascular KAPOSI SARCOMA, Soft Tissue Tumors Fibrous Tumors, etc. FIBROSARCOMA -deep-seated, young, mid-age adult -retroperitoneum, thigh, knee -unencapsulated, infiltrative -all degrees of diff'tion -can be in HERRINGBONE pattern -recur in 50%, METS ᡑ% -35% 5 yr survival INFANTILE- ɚyo, often congenital -extremities, smaller, superficial -85% 5yr survival, Soft Tissue Tumors Fibrous Tumors, etc. DEEP-SEATED FIBROMATOSES (DESMOID TUMORS) -teens, 30s extra-abd-M=W, shoulder, chest abd-ant wall during/after PREG intra-abd-mesentery/pelvic walls -often w/ GARDNER SYNDROME CLINICAL-disfiguring, painful Tx: TAMOXIFEN in some, CTX, XRT -if "mets", probably fibrosarcoma, NODULAR FASCIITIS-most common =infitrative/pseudosarcomatous -reactive to trauma -ADULTS-volar forearm>chest>back -over a couple weeks -after trauma in 10-15% pts MORPH-deep dermis, fascia, subC, mm -abundant mitoses, nucleoli -myofibroblasts, fibroblasts r/o fibromatosis, spindle cell sarc -doesn't recur after excision -self-limiting course Reactive Pseudosarcomatous Proliferations -NOT tumors -plump fibroblasts -RAPID PROLIFERATIVE FASCIITIS PROLIFERATIVE MYOSITIS -in older pts' trunk, prox limbs -large, round prolif fibroblasts -nucleoli -resemble GANGLION cells, Soft Tissue Tumors Skeletal Muscle -almost all malignant Rhabdomyoma -VERY RARE CARDIAC RHABDOMYOMA -prob hamartomatous, Soft Tissue Tumors Smooth Muscle LEIOMYOMA-teens, young adult -often in uterus -most common neo in women -arrector pili mm-skin, nipples, scrotum, labia(genital leios) (pilar leiomyomas)-PAINFUL -multiplicity-AD trait Genital-rare, larger than PILO Piloleiomyoma-limbs/trunk, MALE AD inheritance in family Angioleiomyoma--subQ or dermis -FEMALE -PAIN in 50% MORPH-spindle cells at rt-angles, NODULAR FASCIITIS-most common =infitrative/pseudosarcomatous -reactive to trauma -ADULTS-volar forearm>chest>back -over a couple weeks -after trauma in 10-15% pts MORPH-deep dermis, fascia, subC, mm -abundant mitoses, nucleoli -myofibroblasts, fibroblasts r/o fibromatosis, spindle cell sarc -doesn't recur after excision -self-limiting course Reactive Pseudosarcomatous Proliferations -NOT tumors -plump fibroblasts -RAPID MYOSITIS OSSIFICANS -metaplastic bone present -athletic teens, adults -after trauma in 50% -in SubQ, mm. of prox limbs Sx-swollen/painful->firm, painless MORPH-center w/ fibroblasts ->move out to 'blasts->trabec ->eventually ossifies completely ->marrow grows in Tx-removal is curative, Soft Tissue Tumors SYNOVIAL SARCOMA Cell of Origin Unknown % intra-articular 10% of all soft-tissue sarcomas -20s-40s LARGE JOINTS, esp lower -sometimes parapharyngeal, abd wall MORPH-most are monophasic -can mimic fibrosarcoma -biphasic: epith-like & spindle cells -calcified concretions - XRay -immunohisto-keratin, epith memb Ag Genes: t(x;18), fused (SYT-SSX) Poor Px; METS to LNs, lung, skeleton, Soft Tissue Tumors Smooth Muscle LEIOMYOSARCOMA-adults, elderly -10-20% soft-tissue sarcs -from PILAR ARRECTOR mm. in skin -WOMEN>men -skin, deep tissues of limbs, retroperitoneum -FIRM, PAINLESS, spindle cells IF-vimentin, actin, desmin Px-superficial-good retroperit-large-bad Px -local extension, METS, Soft Tissue Tumors Skeletal Muscle -almost all malignant RHABDOMYOSARCOMA -most common STT in kids, teens - ងyo -H&N, GU (not really NL sk mm) -or in limbs MORPH-rhabdomyoblast in all types -STRAP CELLS -IF: vimentin, actin, desmin,myoglobin -Embryonal-66%-Sarcoma Botryoides yo - grape-like, striations present -nasal cavity, orbit, middle ear, prostate -sheets of cells in myxoid stroma -S.B w/ best Px of RMSs -Alveolar-early-mid teens in limb deep mm. -looks like pulm alveolae -striations in 25% -t(2;13)->PAX3-FKHR->mm. dysregulation, t(1;13) -WORST Px -Pleomorphic-can resemble malig fib hist -ADULTS, in deep soft tissue, Soft Tissue Tumors Fibrous Tumors, etc. NODULAR FASCIITIS-most common =infitrative/pseudosarcomatous -reactive to trauma -ADULTS-volar forearm>chest>back -over a couple weeks -after trauma in 10-15% pts MORPH-deep dermis, fascia, subC, mm -abundant mitoses, nucleoli -myofibroblasts, fibroblasts r/o fibromatosis, spindle cell sarc -doesn't recur after excision -self-limiting course, Soft Tissue Tumors Fibrohistiocytic Tumors BENIGN FIBROUS HISTIOCYTOMA DERMATOFIBROMA-most of BFHs -bland spindle cells, STORIFORM -hyperpigmented skin -persist for yrs, can regress spon'ly -factor XIII-immunohisto SCLEROSING HEMANGIOMA -blood vessels, hemosiderin deps HISTIOCYTOMAS-foamy histiocytes -pretty common-present as adult -dermis, SubQ -painless, slow-growing -ALL infiltrative but not up to epidermis