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This Concept Map, created with IHMC CmapTools, has information related to: Bone II, Joints, ???? Joints JUVENILE RHEUMATOID ARTH (Still Dz) - pretty common - 1-3yo GIRLS>boys -preceded by acute febrile illness -periarticular swelling, big LNs, hep'splen'meg, growth retardation -can->paricarditis, other fibroses DIFF FROM ADULT RA: -OLIGOarthritis more common -large jts before small, ANA (+) -knees, wrists, elbows -NO RF, nodules -variable, may resolve, Bone Tumors, Joints Cartilage-Forming Tumors CHONDROBLASTOMA-rare -TEENS MALE>female-KNEES -epiphyses, apophyses (iliac crest) -pelvis, ribs in older ppl MORPH-sheets of small cells -hyperlob nuc, longit grooves -chicken-wire mineralization -GIANT cells scattered thruout -hemorr cystic degeneration PAINFUL, jt effusions, low mobility -geographic XR lucency, spotty -pulm METS mostly if already had Fx, Bone Tumors, Joints Fibrous, Fibro-osseous FIBROUS CORTICAL DEFECT -very common-30-50% ɮyo kids -devel defects, not neoplasms -metaphysis-distal FEMUR, prox tibia -almost 1/2 bilateral, mult NONOSSIFYING FIBROMA -FCDs > 6cm-detect as teen MORPH-sharp lucencies, sclerosis zone -fibroblasts (in pinwheel), histiocytes(giant cell or foamy) Resolution-spontaneous in few years -few -> fibromas -can -> Fx, Bone Tumors, Joints Fibrous, Fibro-osseous FIBROSARCOMA-M=F -mostly low-intermed grade MALIGNANT FIBROUS HISTIOCYTOMA -MEN>fm -spindled fibs + multinuc GIANT CELLS -any age, MIDDLE-AGED, ELDERLY -metaphysis LONG bones, pelvic bones -usually de novo, some 2ndary: -prev. benign tissue, pagetic bone, irradiated tissue MORPH-destroy underlying tissue CLINICAL-often pathologic Fx -Px-poor if high-grade., GOUT-follows hyperuricemia (not always) -most -> urate nephropathy Risk Fx-Age, genetic (HGPRT), EtOH, obesity, Drugs (thiazides), Lead -synovial fluid a poorer solvent than plasma -attract WBCs, fix complement->LTB4, free rads, neutrophil enzs->injury -TOPHI form in inflamed synovium -PATHOGNOMONIC, also in ligas, tendons, other soft tissues, superficial->ulcers - negatively birefringent (yellow) MORPH-Acute arth->chronic->tophi->kidney -chronic via repetitive ppt->crust (-)Sx hyperuric-puberty in M, menopause F ->acute arthritis-sudden PAIN, warmth, tenderness, no constit Sx (some fever) -50% in 1st metatarsophal jt in 1st attack ->instep, ankle, heel, knee, wrist, finger, elbow -hrs-wks unTx, then complete resolution ->(-)Sx intercritical period ->most recur w/in mos-yrs, then shorter intervals ->then incompelte resolution -CHRONIC TOPHACEOUS GOUT after 12 yrs Px-20% die of renal failure PSEUDO Ca PYROPHOSPHATE CRYSTAL DEP DZ =CPPD=PSEUDOGOUT=chondrocalcinosis -in 30-60% of ᢍyo -2ndary Ca-pyrophosphate dep into jt fluid -weakly birefringent -due to: DM, Wilson Dz, Hypothyroid, hypoMg -hyperPTH, hemochromatosis, ochronosis -in articular matrix, menisci, IV discs -rupture->inflam in joint Px-50% w/ significant joint dmg, supportive Tx, Bone Tumors, Joints Misc Bone Tumors Oncogenes t(11;22)(q24:q12)- 85% - EWS-FL11 fusion t(21;21)(q21;q12) in 5-10%; t(7;22) in ə% - c-myc oncogene EWING SARCOMA -small round cell (like lymphos) tumor of bone -resembles oat cell, rhabdo, neuroblastoma, lymphoma -UNDIFFERENTIATED -YOUNGEST AT PRESENTATION-10-15yo -BOYS>girls, WHITES>blacks -FEMUR, flat pelvis, painful, warm, swollen -high ESR, leukocytosis, anemia -ONION-SKIN reactive bone deposition MORPH-medullary cavity->periosteum -HOMER-WRIGHT ROSETTES, few mitoses Tx: CTX+ surgery+/-XRT - 50% survival PRIMITIVE NEUROECTODERMAL TUMOR -similar to Ewing-BUT neural diff'tion, GOUT-follows hyperuricemia (not always) -most -> urate nephropathy Risk Fx-Age, genetic (HGPRT), EtOH, obesity, Drugs (thiazides), Lead -synovial fluid a poorer solvent than plasma -attract WBCs, fix complement->LTB4, free rads, neutrophil enzs->injury -TOPHI form in inflamed synovium -PATHOGNOMONIC, also in ligas, tendons, other soft tissues, superficial->ulcers - negatively birefringent (yellow) MORPH-Acute arth->chronic->tophi->kidney -chronic via repetitive ppt->crust (-)Sx hyperuric-puberty in M, menopause F ->acute arthritis-sudden PAIN, warmth, tenderness, no constit Sx (some fever) -50% in 1st metatarsophal jt in 1st attack ->instep, ankle, heel, knee, wrist, finger, elbow -hrs-wks unTx, then complete resolution ->(-)Sx intercritical period ->most recur w/in mos-yrs, then shorter intervals ->then incompelte resolution -CHRONIC TOPHACEOUS GOUT after 12 yrs Px-20% die of renal failure Primary vs Secondary Secondary-10% LEUKEMIAS-inc'd cell turnover CHRONIC RENAL DZ-dec'd excretion IEM-complete HGPRT def-X-linked LESCH-NYHAN SYNDROME ->increased de novo purine synth ->neuro deficits, SEVERE PAIN, self-mutilation, gouty arthritis, GOUT-follows hyperuricemia (not always) -most -> urate nephropathy Risk Fx-Age, genetic (HGPRT), EtOH, obesity, Drugs (thiazides), Lead -synovial fluid a poorer solvent than plasma -attract WBCs, fix complement->LTB4, free rads, neutrophil enzs->injury -TOPHI form in inflamed synovium -PATHOGNOMONIC, also in ligas, tendons, other soft tissues, superficial->ulcers - negatively birefringent (yellow) MORPH-Acute arth->chronic->tophi->kidney -chronic via repetitive ppt->crust (-)Sx hyperuric-puberty in M, menopause F ->acute arthritis-sudden PAIN, warmth, tenderness, no constit Sx (some fever) -50% in 1st metatarsophal jt in 1st attack ->instep, ankle, heel, knee, wrist, finger, elbow -hrs-wks unTx, then complete resolution ->(-)Sx intercritical period ->most recur w/in mos-yrs, then shorter intervals ->then incompelte resolution -CHRONIC TOPHACEOUS GOUT after 12 yrs Px-20% die of renal failure Primary vs Secondary Primary-90% ENZYME DEFECTS ->overproduction, most w/ increased excretion Unknown-90% Partial HGPRT def-10% -overproduction of uric acid, Bone Tumors, Joints Cartilage-Forming Tumors CHONDROMYXOID FIBROMA -RAREST -teens, 20s, MALE -metaphyses of long bones -3-8cc-hyaline cart, myxoid -fibrous septae -some calcification, GIANT cells -may recur, but no mets, malig, Bone Tumors, Joints Misc Bone Tumors GIANT CELL TUMOR=osteoclastoma -20s-40s yo -locally aggressive-epi-, metaphyses -teens just metaphyses (due to GP) -mostly around KNEE, joints ->feels arthritic, pathologic Fx -most SOLITARY, purely LYTIC, EXPANSILE -destroy overlying cortex->reactive bone -monocyte-mac lineage MORPH-large, red-brown, cystic -MONONUCS proliferate-syncytial -frequent MITOSES -bkgrd of GIANT cells (same nuclei as mononucs-Dx) -R/O brown tumor, etc. Px- <=4% METS TO LUNGS ->malignant if IRRADIATED, CHONDROMAS-15-45yo ɛcm -intraosseous hyaline cart -enchondroma-in medulla -most common-short tubular bones -hands, feet -solitary, metaphysis OLLIER DZ-enchondromatoses ->more freq sarcomas MAFFUCCI syn-plus hemangiomas ->OV CA, brain GLIOMAS ->CHONDROSARCOMA -or subperiosteal, juxtacortical -via rest of growth plate cart MORPH-endochon ossification at periph of lesions->calcifies, dies -can ->pathologic Fx, pain -Xray-O RING SING-oval lucencies Risk w/ MARFUCCI CHONDROSARCOMA ᡠs MEN - AXIAL SKELETON -RARELY DISTAL EXTREMITIES -2nd most common (1-osteosarc) -many from ENDOCHONDROMA -grade 3=chondroblastic OS -10% of lo-grade w/ hi-grade lesion Clear Cell Chondrosarcoma- -many giant cells, bone formation (looks like osteosarcoma) -epiphyses of long bones Mesenchymal-hyaline islands -small round cells surround CLINICAL-central skel-pelv, should, ribs -higher grade->more LUCENT -METS to lungs, skeleton Tx: CTX, surgical excision pX: GOOD IF GRADE 1, 2, Bone Tumors, Joints Misc Bone Tumors MULTIPLE MYELOMA -most common bone neoplasm -multicentric, involves marrow diffusely -VERTEBRAL BONE MORPH-round lesions, w/ soft reddish material -full of plasma cells, ???? Joints Tumors-Villonodular Synovitis-benign -actually clonal and neoplastic -20s-40s, M=F -excise surgically, both often recur GIANT CELL TUMOR OF TENDON SHEATH -localized nodular tenosynovitis -well-circumscribed -solitary, slow-growing, painLESS -wrist, finger tendon sheaths -most common mesen tumor of hand PIGMENTED VILLONODULAR SYNOVITIS -brown (hemosiderin), foamy macs, giant cells, sclerotic zones -monoarticular arthritis in 80% -KNEE, hip, ankle, calcaneocuboid jts -pain, locking, swelling -can be palpated, can invade soft tissue, Bone Tumors, Joints Fibrous, Fibro-osseous FIBROUS DYSPLASIA -localized developmental arrest Monostotic (one bone)-70%-M=F -early teens, stops at GP closure -1/3 CRANIOFACIAL, 1/3 RIBS -1/3 FEMUR/TIBIA -(-)Sx or disfigurement Polyostotic (many bones)-27% -femur, skull, tib, hum, ribs, fib -often CRANIOFACIAL, s/p girdles McCune-Albright Syndrome-3% -somatic mutation, GIRLS>boys -polyostotic dz-w/ CAFE AU LAIT, precocious puberty, endocrine -unlateral problems MORPH-disorganized GP-hyal cart, fibs -cysts, hemorr, foamy macs -curvy, chinese letter trabeculae CLINICAL-ground-glass X-ray -rarely->SARCOMA, malignancy -inc'd risk if IRRADIATION, Bone Tumors, Joints Cartilage-Forming Tumors CHONDROSARCOMA ᡠs MEN - AXIAL SKELETON -RARELY DISTAL EXTREMITIES -2nd most common (1-osteosarc) -many from ENDOCHONDROMA -grade 3=chondroblastic OS -10% of lo-grade w/ hi-grade lesion Clear Cell Chondrosarcoma- -many giant cells, bone formation (looks like osteosarcoma) -epiphyses of long bones Mesenchymal-hyaline islands -small round cells surround CLINICAL-central skel-pelv, should, ribs -higher grade->more LUCENT -METS to lungs, skeleton Tx: CTX, surgical excision pX: GOOD IF GRADE 1, 2, ???? Joints GOUT-follows hyperuricemia (not always) -most -> urate nephropathy Risk Fx-Age, genetic (HGPRT), EtOH, obesity, Drugs (thiazides), Lead -synovial fluid a poorer solvent than plasma -attract WBCs, fix complement->LTB4, free rads, neutrophil enzs->injury -TOPHI form in inflamed synovium -PATHOGNOMONIC, also in ligas, tendons, other soft tissues, superficial->ulcers - negatively birefringent (yellow) MORPH-Acute arth->chronic->tophi->kidney -chronic via repetitive ppt->crust (-)Sx hyperuric-puberty in M, menopause F ->acute arthritis-sudden PAIN, warmth, tenderness, no constit Sx (some fever) -50% in 1st metatarsophal jt in 1st attack ->instep, ankle, heel, knee, wrist, finger, elbow -hrs-wks unTx, then complete resolution ->(-)Sx intercritical period ->most recur w/in mos-yrs, then shorter intervals ->then incompelte resolution -CHRONIC TOPHACEOUS GOUT after 12 yrs Px-20% die of renal failure, ???? Joints Seroneg Spondyloarthropathies ANKYLOSING SPONDYLOARTHRITIS -Marie-Strumpell Dz, Rheum Spon -YOUNG MEN-HLA-B27 (90%) -ass'd w/ IBD -sacroiliac jts, spine REITER SYNDROME 1. Reactive Arthritis + 2. Nongonococcal Urethr/Cervicitis + 3. Conjunctivitis Via-Shig, Salmonella, Yersinia, Campylo, Chlamydia IBD ARTHRITIS -in 25% of UC pts -resolves w/ Tx of UC -NO pannus ENTEROPATHIC ARTHRITIS-bowel infx -Yersinia, Salmonella, Shigella, Campylo -all w/ LPS -HLA-B27, abrupt onset in knees, ankles PSORIATIC ARTHRITIS-no RF, 5% of psor -HLA-B27 - 35-45yo -DIP jts first, asymmetric ->SAUSAGE FINGER-inflam digital tendon -not as bad as RA-slow, but acute in 1/3, ???? Joints More infectious: TUBERCULOUS-insidious, progressive -granulomas w/ caseous necrosis -synovium can->pannus->erosion -can->ankylosis -wt-bearing joints-hips, knees,ank LYME-Borrelia burgdorferi -wks-2yrs to jts in 80% -remitting, migratory-1-2jts/episode -dominant feature of late dz -episodes last wks-mos -papillary synovitis -onion-skin aa. walls VIRAL-parv B19, rubella, HCV -many arthritides found in HIV, Bone Tumors, Joints Cartilage-Forming Tumors OSTEOCHONDROMA=Exostosis -1-20cm - TEENS, 20s for solitary -in mult hered exostosis-AD in younger -Chrom 8, 11, 19 -bowed, shortened bones ->inc. risk for CHONDROSARCOMA -only in endochondral bones ->diag from long axis -METAPHYSIS near physis - tubular -KNEE, pelvis, scapula, ribs-sessile MENɯx women MOPRH-mushroom-shaped -outer layer-benign hyaline cartilage -medulla is continuous w/ bone -stop growing at bone plate closure -rarely -> sarcoma (esp if MHE), OSTEOCHONDROMA=Exostosis -1-20cm - TEENS, 20s for solitary -in mult hered exostosis-AD in younger -Chrom 8, 11, 19 -bowed, shortened bones ->inc. risk for CHONDROSARCOMA -only in endochondral bones ->diag from long axis -METAPHYSIS near physis - tubular -KNEE, pelvis, scapula, ribs-sessile MENɯx women MOPRH-mushroom-shaped -outer layer-benign hyaline cartilage -medulla is continuous w/ bone -stop growing at bone plate closure -rarely -> sarcoma (esp if MHE) Risk if Mult Hereditary Exostosis CHONDROSARCOMA ᡠs MEN - AXIAL SKELETON -RARELY DISTAL EXTREMITIES -2nd most common (1-osteosarc) -many from ENDOCHONDROMA -grade 3=chondroblastic OS -10% of lo-grade w/ hi-grade lesion Clear Cell Chondrosarcoma- -many giant cells, bone formation (looks like osteosarcoma) -epiphyses of long bones Mesenchymal-hyaline islands -small round cells surround CLINICAL-central skel-pelv, should, ribs -higher grade->more LUCENT -METS to lungs, skeleton Tx: CTX, surgical excision pX: GOOD IF GRADE 1, 2