Bone Tumors

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Bone Tumors
2011-04-26 15:14:02
Bone Tumors

Overview of bone tumor pathology for M2 musculoskeletal course at UNMC
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  1. Primary bone tumor
    • Common <30 yr
    • common around knee, humerous
    • can arise any cell in bone-marrow 40%, osteoblast 19%, osteoclast 10%, carilage 22%, fibroblast
    • most common site-prox femur, distal tibia, prox humerous
    • young: benign=ostechchrondroma, malig=osteosarcoma
    • older: benign-osteoma, malig-mult myeloma
  2. Secondary tumors
    • PTBLK- prostate-blast, thyroid, breast-blast, lung, kidney
    • kids: neuroblastoama, rhabdomyosarcoma, retinoblastoma
    • mult lesion, or history of cancer=mets
  3. Clinical
    • Benign= ASx/ indicental
    • pain-nonspecific but sometimes helpful, typically deep, contant, worse at night, wks to months
    • mass complicated by a pathologic fracture, growing lesion, significan ST rxn
    • swelling-usu late or large tumor, may signify ST involvement
  4. Radiographic evaluation
    • Plan XR= usually first imaging, can predict probable histology
    • CT- useful when plain film is difficult due to anatomic site
    • MRI- choice for local staging and surgery planning NOT Dx
  5. Biopsy
    • May or may not be needed
    • need enough tissue to Dx and grade
    • Hx of Multiple Myeloma or prior cancer w/mult bony lesion
    • ultimate Dx of bone tumor require radio-histo correlation
  6. Benign primary tumors
    • Not all are neoplastic-hamartomas, disordered growth, often incidental, XR usually pathognomic
    • osteochroma, osteoid oseoma, endochromcoma, fibrous, dysplasia, giant cell tumor of bone, aneursymal bone cyst, non—ossyfying fibroma, eosinophicli granuloma
  7. Benign Primary Tumor Tx
  8. Osteochondroma
    • Most common benign
    • mushroom shaped, bone w/cartilage cap
    • located at epiphyseal line
    • grows w/puberty, stop after plate close
    • usually solitary
    • mult- humt hereditary exostosis syndrom
    • Tx: excision at base if Sx
  9. Osteoid osteom
    • Reactive, very rare
    • <20 y.o.
    • deep seeded bone pain, VERY sensitive to NSAIDS-PGE2 release
    • like diaphysis of long bones
    • small tumor focus=nidus causes abundant reactive bone formation
    • Tx: conservative, RFA-ablation
  10. Chondroma
    • Childhood, persist throughout life-remnants of cartilage that failed to ossify in growth
    • junction of diaphysis and metaphysis in long bones or short tubular bones of HAND & FOOT
    • collection of benign hyaline cartilage
    • often incidental
    • usually solitary
    • syndrome-olliers, affucci
    • Tx: conservative
  11. Fibrous dysplasia
    • Development abnormality-failure of primitive bone to align along stress lines-weaker bone susceptible to deformatives/fractures
    • usually <30 y.o.
    • prox femur, jaws, ribs
    • ground glass
    • mono or polyosticic
    • McCune-albright synd
    • histo: Chinese letter-irreg bone trabeulae, in fibrous stroma
    • Tx: conservative
  12. McCune-Albright
    Polyostic fibrous dyplasia, café au lait, endocrine abnormalities esp precocious puberty
  13. Giant cell tumor
    • Young pts
    • like epiphysis of long bones or vertebral bodies
    • knee pain, ABC change or pathologic fracture
    • radiology: soap bubble lesion-multicystic w/sclerotic rim, crosses epiphyseal line
    • Tx: curettage-high recurrece rate, joint replacement if gets into space
  14. Aneurismal bone cyst
    Unknown cause-primary of secondary
  15. Non-ossifying fibroma
    • Metaphyseal fibrous defect
    • common-most incidental finding
    • error remodeling metaphyseal corticies in growth
    • eccentric lesion in metaphyseal cortex or prox/distal tibia and fibula
    • round or oval defects w/sclerolitc rim
    • Histo: swirling of bland spindle cells w/admixed osteoclasts
    • Tx: reassurance
  16. Langerhand cell histiocytosis
    • Eosinophilic granuloma
    • tumor of langerhan cells-related to macrophages
    • occurs in mult organs, bone in kids
    • usually incidental, may have mild pain or fracture
    • great radiology imitator-looks like malignant esp ES/PNET
    • Tx: observation, biopsy usually curative, low dose radiation
    • CD-CD1a, S100+
    • EM: berbeck granules
  17. LCH variants
    • Hand-schuller Christian: (unisystem multifocal- exophtalmus, diabetes insipidus, lytic skull lesion)
    • letterer-siwer: multisystem multifocal- young infants/toddlers, nearly always fatal
  18. Osteosarcoma
    • Tumor of osteoblasts-tumor osteoid
    • most common primary bone malig in kids
    • may be primary or secondary
    • secondary- ADULTS, arises in pagetic bone, radiated bone-esp w/alkylating agents
    • distal femur, prox tibia, prox humerus, usu in metaphysis, usually unifocal, 20% have mets at presentation
    • clinical- pain, soft tissue mass for months, pathological fracture
  19. Ostesarc dx
    • Radiology- aggressive, usu radiodense lesion=osteoid
    • periosteal reaction- not specific response to insult, codman’s triangle, sunburnt pattern
    • seen in lesion that rapidly grow- periosteum can’t lay down new bone fast enough to form organized cortex and ossifies irreg
  20. Osteosarc gross
    • Gritty and calcified
    • destruction of bony arch
    • usually involves surrounding tissue
  21. Osteosarc histo
    • Atypical spindle cells that produce abnormal woven osteoid
    • osteoid in ST lesion is definitioal of osteosarc
  22. Osteosarc variants
    Conventional 80%, periosteal, chondroblastic, periosteal, telangectasic, small cell, dediff
  23. Osteosarc Tx
    • Xray and incisional Bx for dx w/MRI
    • Neoadjuvant chemo and repeat MRI, then resection w/wide margins-limb salvage better
    • poor prog: <90% tumor response, large, adult or secondary, axial skeletal site
    • 5 yr survival 70%
  24. Chondrosarcoma
    • Malig tumor of chrondrocytes
    • older pts than Osteosarcoma-30-60 yr
    • usually large tumor at Dx
    • most common in trunk/ prox limgs
    • pelvis, shoulder, prox humerus/femur-trunk and prox limbs
    • Xray: stippled/popcorn caliciction w/ST involvement
  25. Chondrsarcoma gross/hist
    • Lobular tumor w/firm grayish cartilage-NO OSTEOID
    • hyaline cartilage but more cellular w/atypia
    • grade important for prognosis
  26. Chondosarcoma Tx
    • Varies
    • tx: surgery-chondrocyte don’t respond well to chemo
    • prognosis highly dep on grade
  27. Ewing sarcoma/primitive neuroectoderm
    • SRBCT
    • 2nd and 3rd decades
    • pelvis, LE, spine
    • Askin tumor: ES/PNET involving rib
    • diaphysis
    • Histo: can see pseudorosettes w/neuritic processes in center
    • CD99 pos
    • translocation: t(11;22)
    • Tx: neoadjuvant chemo and surgery
    • 5 yr survival 65%
    • radiation for close margins