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Musculoskeletal Tumors

AB
ChondrosarcomaOlder males
ChondrosarcomaCentral location
ChondrosarcomaLarge destructive mass
ChondrosarcomaGlistening white tissue
ChondrosarcomaNo osteoid
OsteochondromaExostosis
OsteochondromaProtruding “mushroom”
OsteochondromaEnchondral bones only
OsteochondromaMetaphysis near growth plate (knee)
OsteochondromaYoung males
OsteochondromaEXT gene inactivation in growth plate chondrocytes
OsteosarcomaOsteoid formation by malignant cells
OsteosarcomaTeenage boys, knee metaphysis
OsteosarcomaRB gene (13q14) in 60%
Osteosarcoma2nd peak in older adults with abnormal bone (Paget disease)
ChondroblastomaChondroblastoma
ChondroblastomaTennage boy, knee, Epiphysis
ChondroblastomaMonotonous small Chondroblasts
ChondroblastomaLacelike matrix calcifies (chicken wire)
ChondroblastomaRecur locally
ChondroblastomaRare lung metastasis à Curettage emboli
Giant cell Tumor of boneGiant cell tumor of bone
Giant cell Tumor of boneEpiphysis (starts in metaphysic, where it stays if epiphyseal plate still present)
Giant cell Tumor of boneYoung adults, knee
Giant cell Tumor of boneMononuclear Cell is proliferative component
Giant cell Tumor of boneFuse to form osteoclast-like giant cells (osetoclastoma)
Giant cell Tumor of boneBenign but locally agressive
Chondromyxoid fibromaMistaken for sarcoma
Chondromyxoid fibromaTeen/ y.a male; metaphysis of long bones
Chondromyxoid fibromaHyaline cartilage, fribrous septa, and myxoid regions with stellate cells; can show cytologic atypia
Chondromyxoid fibromaMay recur, never malignant
osteoid osteomaMales 10-30 ;Lower limb
osteoid osteoma< 2cm
osteoid osteomaPain, nocturnal
osteoid osteomaProstaglandin E2
osteoid osteomaAspirin helps
osteoid osteomaRadiolucent nidus with sclerotic border
osteoblastomaMales: 10-30; Spine and skull
osteoblastomaLarger than 2 cm
osteoblastomaLess specific pain
osteoblastomaNo PGE2
osteoblastomaAspirin doesn’t help
osteoblastomaRadiolucent nidus with sclerotic border
Ewing Sarcoma and PNETSmall round blue cell tumor
Ewing Sarcoma and PNETTeens; Diaphysis (Medullary cavity)
Ewing Sarcoma and PNETSystemic symptoms (fever)
Ewing Sarcoma and PNETT(11;22): results in fusion gene, a dominant oncogene leading to cell proliferations
PNETshows neural differentiation
EnchodromaYoung Adults; short tubular bones of hands and feet
EnchodromaBenign cartilage
EnchodromaProbably arise from cartilaginous rests
EnchodromaMultiple = Ollier disease
EnchodromaMultiple + hemangiomas = Maffucci syndrome
EnchodromaChondroma – bone surface, less common
Fibrous dysplasiaYA, Adolescents; Benign intramedullary mass-like lesion
Fibrous dysplasiaCurvilinear trabeculae (Chinese characters) of woven bone; no mature osteoblasts; cellular fibroblastic stroma
Fibrous dysplasiaMonostotic > Polyostotic > McCune-Albright (café au lait spots and endocrinopathies
Fibrous dysplasiaPostzygotic, somatic mutation for G-protein à increased activity of cAMP à increase cell function; distribution of mutation determines degree
Pagets DiseaseMosaic pattern – prominent irregular cement lines
Pagets DiseaseStages: osteoclastic > mixed > osteoblastic > quiescent (osteosclerotic)
Pagets DiseasePossibly viral; paramyxovirus (measles)
Pagets DiseasePolyostotic >>> monostotic
Pagets DiseaseHypervascular à hear failure
Pagets DiseaseSecondary sarcoma
GoutMonosodium urate crystal deposition
GoutPurine metabolism (usually unknown defect; salvage pathway: HGPRT)
GoutCrystals precipitate in joints à activate inflammation
GoutTophi in joints, surrounding soft tissue
GoutAcute and chronic arthritis; nephropathy
Ganglio (cyst)Wrist, usually dorsum
Ganglio (cyst)Near tenosynovium or joint capsule
Ganglio (cyst)Contains thick fluid similar to synovial
Ganglio (cyst)No true cell lining (synovial or otherwise)
Ganglio (cyst)Degenerative vs. synovial-related (traumatic?)
Giant cell tumor of tendon sheathJoint equivalent is PVNS (usually knee)
Giant cell tumor of tendon sheathMost common mesenchymal neoplasm of hand
Giant cell tumor of tendon sheathSmaller cells are proliferative component
Giant cell tumor of tendon sheathGiant cells variable in prominence
Giant cell tumor of tendon sheathVariegated (hemosiderin = brown; foamy macrophages = yellow)
Superficial FibromatosesPalmar (Dupuytren’s), plantar, and penile (Peyronie’s)
Superficial FibromatosesM>F
Superficial FibromatosesHypercellular (Myofibrobroblasts?)
Superficial FibromatosesNot aggressive
Deep FibromatosesDesmoid tumor
Deep FibromatosesAbdominal ones related to pregnancy
Deep FibromatosesExtra/intra-abdomical
Deep FibromatosesLocally aggressive
Deep FibromatosesDifficult to excise
Myositis ossificansOssified soft tissue mass
Myositis ossificansProximal extremities, young, athletic
Myositis ossificansUsually post trauma
Myositis ossificansZonation: central fibroblastic zone maturing peripherally to trabecular bone
Myositis ossificansDifferential: extraskeletal osteosarcoma (no zonation, older patients)
Nodular FasciitisNon-ossified soft tissue mass
Nodular FasciitisForearm
Nodular FasciitisHistory of trauma uncommon
Nodular FasciitisMitotically active cellular proliferation of fibroblasts (“pseudosarcoma”)
Malignant fibrous histiocytomaHas giant cells
Cytokeratin:usually a marker of epithelial cell
Synovial SarcomaBad prognosis
Synovial SarcomaHas cytokeratin
Synovial SarcomaBiphasic: spindled and epithelial-like
Synovial SarcomaT(x;18)
Synovial SarcomaUsually near joints of limbs, but not directly from synovium
Synovial SarcomaAdolescents and young adults, M>F



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