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Primary Tumors Of Bone
 

Primary Tumors Of Bone

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    Primary Tumors Of Bone Primary Tumors Of Bone Presentation Transcript

    • Primary tumors of bone Mr Sreeram Penna www.eyst.org
    • Malignant Tumors of Bone
      • Osteosarcoma
      • Chondrosarcoma
      • Ewing Sarcoma
      • Chordoma
      • Adamantinoma
      • Malignant Vascular tumors
      • Malignant fibrous histocytoma and fibrosarcoma
      • Multiple myeloma, Plasmacytoma and Lymphoma
      • Metastatic bone tumors
      www.eyst.org
    • Benign/Aggressive tumors
      • Giant cell tumor
      • Chondroblastoma
      • Chondromyxoid fibroma
      • Osteoblastoma
      • Langerhans cell histiocytosis
      www.eyst.org
    • Benign tumors
      • Bone-forming
      • Osteoid Osteoma
      • Bone island
      • Cartilage Lesions
      • Chondroma
      • Osteochondroma
      • Fibrous lesions
      • Non ossifying fibroma
      • Cortical desmoid
      • Benign fibrous histocytoma
      • Fibrous displasia
      • Osteofibrous dysplasia
      • Desmoplastic fibroma
      www.eyst.org
    • Benign tumors cont
      • Cystic Lesions
      • Unicameral bone cyst
      • Aneurismal bone cyst
      • Intraosseous ganglion cyst
      • Epideromoid cyst
      • Fatty tumors
      • Lipoma
      • Vascular tumors
      • Hemangioma
      www.eyst.org
    • Non neoplastic conditions
      • Pagets disease
      • Brown tumor of hyperparathyroidism
      • Bone infarct
      • Osteomyelitis
      • Stress fracture
      • Posttraumatic osteolysis
      www.eyst.org
    • Principles of mx - Investigations
      • Plain radiographs of the bone
      • CT scans
      • MRI
      • Bone scans
      • Chest X-ray
      • CT of chest
      www.eyst.org
    • Grading
      • Done histologically
      • Biopsy need to be performed by team prepared to do the resection.
      www.eyst.org
    • Osteosarcoma
      • Tumor characterised by production of osteoid by malignant cells.
      • 2 nd most commonest primary bone tumor, 20% primary malignancies,1-3 per 1 million population.
      • Age: Primary high grade osteosarcoma – 10 – 20 yrs , Parosteal osteosarcoma – 3 rd and 4 th decade, Secondary osteosarcoma in older population
      • M>F (except parosteal osteosarcoma)
      • Genetic association: Rothmund-Thompson syndrome, Li-Fraumani syndrome, hereditary form of retinoblastoma.
      • Location: distal femur, proximal tibia, and proximal humerus.
      www.eyst.org
    • Osteosarcoma
      • Symptoms: Progressive pain, night pain or painless mass.
      • Mostly metaphyseal, 10% diaphyseal, 1% epiphyseal
      • Classification :
      • - Primary Osteosarcoma:
      • conventional
      • low grade intramedullary
      • parosteal
      • periosteal
      • high grade surface,
      • telangectic
      • small cell
      • - Secondary Osteosarcoma
      www.eyst.org
    • Conventional Osteosarcoma
      • High grade
      • Common type of Osteosarcoma
      • Common radiographic appearance is aggressive lesion producing osteoid matrix. Periosteal reaction may take the form of “codmans triangle” or “sunbrust” or “hair on end” appearence
      www.eyst.org
    • Periosteal Osteosarcoma
      • Intemidate grade
      • Arises from surface of bone
      • Commonly on femur and tibia.
      www.eyst.org
    • Intramedullary Osteosarcoma
      • Rare
      • Low grade
      www.eyst.org
    • Parosteal Osteosarcoma
      • Low grade malignancy
      • Rare
      • Arises on surface of bone and invades medullary cavity only at later stages.
      • It has peculiar tendency to occur as a lobulated mass on the posterior aspect of femur
      www.eyst.org
    • High grade surface Osteosarcoma
      • High grade
      • Least Common
      • Radiographs show invasive lesions with with ill defined borders
      www.eyst.org
    • Telangectic Osteosarcoma
      • Lytic lesion
      www.eyst.org
    • Small cell Osteosarcoma
      • Rare
      • High grade
      • Resemble Ewing sarcoma or Lymphoma
      www.eyst.org
    • Secondary Osteosarcoma
      • These occur at the site of another disease process
      • Older patients
      • E.g. Pagets disease, previous radiation treatment, fibrous dysplasia, bone infarcts, osteochondromas, chronic osteomyelitis, dedifferentiated chondrosarcomas, melorhestosis and osteogenesis imperfecta.
      www.eyst.org
    • Investigations
      • Plain radiographs
      • CT scan
      • Bone scan
      • MRI
      • Chest radiographs and CT for staging
      www.eyst.org
    • Osteosarcoma treatment
      • Neoadjuvant chemotherapy
      • Wide or radical surgery (resection or amputation)
      • Adjuvant chemotherapy
      www.eyst.org
    • Chondrosarcoma
      • 9% of primary malignancies of bone
      • Age: broad, primary chondrosarcoma peak around 40 – 60yrs, secondary chondrosarcoma 25 – 45 yrs.
      • Any location but common around pelvis, proximal femur, proximal humerus.
      • Most common malignancy in hand.
      • Clinically: increasing pain and palpable mass. Pain in absence of pathological fracture is helpful to differentiate between enchondroma and low grade chondrosarcoma
      www.eyst.org
    • Secondary Chondrosarcoma
      • Olliers disease (multiple enchondromatosis)
      • Maffuci syndrome (multiple enchondromatosis + soft tissue haemangioma)
      • Multiple hereditary exostoses
      • Solitary osteochondroma
      • Synovial chondromatosis
      • Chondromyxoid fibroma
      • Periosteal Chondroma
      • Chondroblastoma
      • Previous radiation treatment
      • Firbours dysplasia
      www.eyst.org
    • Chondrosarcoma - xrays
      • Appearence similar to enchondroma, it is a lesion arising in medullary cavity with irregular matrix calcification. Pattern is described as “punctate,” “popcorn,” or “comma-shaped”. Compared with enchondroma, chondrosarcoma has more aggressive appearence with bone destruction and cortical erosions, periosteal reaction, and rarely soft-tissue mass.
      www.eyst.org
    • Mesenchymal chondrosarcoma www.eyst.org
    • Clear cell chondrosarcoma Epiphyseal Gaint Cell Tumor Chondroblastoma Clear cell chondrosarcoma www.eyst.org
    • Dedifferentiated chondrosarcoma Radiographic features of dedifferentiated chondrosarcoma often show a more aggressive radiolucent area juxtaposed on a otherwise typical chondrosarcoma. www.eyst.org
    • Chondrosarcomas Treatment
      • Low grade – Extended curettage with use of intraoperative adjuvant treatment.
      • High grade – Wide or radical resection or amputation.
      • Radiotherapy as palliative for inaccessible lesions.
      www.eyst.org
    • Ewing sarcoma
      • 4 th most common primary malignancy
      • 9% of primary malignancy of bone
      • Age: most occur in 5 – 25 yrs
      • Commonly metaphyses of long bones(often extension to diaphyses) and flat bones of shoulder and pelvic girdle.
      • Males > Females
      • Clinically: Pain(insidious onset, mild and intermittent initially), fever, erythema, swelling.
      • Investigation: elevated ESR, raised CRP
      www.eyst.org
    • Ewing sarcoma – xrays
      • Destructive lesion in the diaphyses of longbone with and “onion skin” periosteal reaction.
      www.eyst.org
    • Ewing sarcoma - MRI
      • To evaluate full extent of lesion
      www.eyst.org
    • Ewing sarcoma - investigations
      • CXR
      • CT chest
      • Bone scan
      • Bone marrow aspirate
      www.eyst.org
    • Ewing sarcoma – treatment
      • Radiosensitive
      • Large central unresectable mass – radiotherapy.
      • Smaller more accessible lesions surgery.
      • Neo adjuvant and adjuvant chemotherapy
      www.eyst.org
    • Chordoma
      • Rare malignant neoplasm arises from notochord remnants.
      • Second most common in spine, most common in sacrum.
      • 50% of chordomas arise in sacrococcygeal region, 30% arise in base of the skull.
      • Age sacrococcygeal 50 – 70 yrs spenooccipital 40 – 60 yrs
      • Clinically: spenooccipital: headaches, symptoms of cranial nerve compression, retropharyngeal abscess. Sacrococcygeal: lower back pain, sciatic pain, bowel and bladder problems. Palpable mass on PR. Spinal: presents with nerve root or cord compression.
      www.eyst.org
    • Chordoma radiology
      • Destructive lesions, virtually arising from midline.
      www.eyst.org
    • Adamantinoma
      • Arises from aberrant epithelial cells.
      • Prediliction to tibia
      • 2 nd and 3 rd decade
      • Clinically pain and palpable mass.
      • Multiple sharply demarcated lesions in tibial diaphyses
      • DD: Osteofibrous dysplasia. (diff by aggressive appearance)
      www.eyst.org
    • Malignant Vascular tumors
      • Hemangioendothelioma – low grade
      • Angiosarcoma – high grade
      www.eyst.org
    • Malignant fibrous histocytoma and fibrosarcoma
      • 3 – 5 % primary bone malignancies
      • All ages except 1 st decade
      • Both sexes affected equally
      • Tendency to occur in distal femur and proximal tibia.
      • 25% considered secondary from paget’s disease, irradiation, giant cell tumor and bone infarction.
      • Clinically presents with pain and pathological fracture(20%)
      • Radiologically aggressive appearance and are typically purely lytic with indistinct borders.
      www.eyst.org
    • Giant Cell Tumor
      • 5% of bone neoplasms
      • Age: 20 to 40 yrs
      • Female>Male
      • Location: distal femur> proximal tibia > distal radius
      • Benign but pulmonary metastasis can occur
      • Clinically: progressive pain, pathological fractures(10 – 30 %)
      • Radiologically lesions are eccentrically located in the epiphysis of long bones and usually abut subchondral bone. Lesions are purely lytic.
      • MRI useful for determining extent of disease.
      www.eyst.org
    • Giant cell tumor – x rays www.eyst.org
    • Giant cell tumor – MRI Dark in T1 weighted image and Bright in T2 weighted images www.eyst.org
    • Giant cell tumor – treatment
      • Aggressive extended curettage and use of adjuvants like argon beam coagulator. And fill the bone cavity with cement or bone graft
      • Inoperable lesions in pelvis use radiation or embolisation.
      • Follow up is very imp as lesions can reccur.
      www.eyst.org
    • Chondroblastoma
      • Age: 10 – 25 yrs
      • M:F is 2:1
      • Commonly distal femur, proximal humerus, and proximal tibia. Older patients flat bones
      • Clinically: progressive pain
      • Radiologically: well circumscribed lesion centered in epiphyses of long bone, or an apophysis like greater trochanter or greater tuberosity. Often it has surrounding rim of reactive bone.
      • In children well circumscribed epiphyseal lesion that crosses open growth plate is diagnostic. In Adults DD is giant cell tumor and clear cell chondrosarcoma.
      • Treatment is extended curettage and adequate follow-up
      www.eyst.org
    • Chondroblastoma - adults www.eyst.org
    • Chondroblastoma - kids www.eyst.org
    • Chondromixoid Fibroma
      • 10 – 30 yrs
      • Commonly proximal tibia
      • Clinically pain or swelling
      • Xrays – well circumscribed lesion with rim of sclerosis in metaphyses of long bone. Intra lesional calcium is absent.
      • Treatment – resection or extended curettage with bone grafting
      www.eyst.org
    • Chondromixoid Fibroma www.eyst.org
    • Osteoblastoma
      • Bone forming neoplasm
      • 10 – 30 yrs old
      • M:F is 3:1
      • Any bone but commonly in spine
      • Clinically: Pain worse at night relived by NSAID, in spine scoliosis or root or cord compression.
      • Xrays: Spine it shows bone forming neoplasm in posterior elements(DD aneurismal bone cyst and Osteoid osteoma). Out side spine calcified central nidus with surrounding radiolucent halo and reactive sclerosis. (DD Osteoid osteoma)
      • Treatment extended curettage or resection. In spine may require instrumented fusion.
      www.eyst.org
    • Osteoblastoma www.eyst.org
    • Langerhans Cell Histiocytosis
      • Also known as Histocytosis X
      • Isolated bony lesions called as eosinophilic granuloma
      • 5 – 20 yrs
      • Clinically progressive pain, fever, local signs of inflammation
      • Commonly affects vertebral bodies, flat bones and diaphyses of long bones.
      • X-rays – in spine vertebra plana. In flat bones lesions are well circumscribed and “punched out” purely lytic lesions. In diaphyses of long bones aggressive permeative appearance with periosteal reactive bone formation.
      • Biopsy is required for diagnosis
      • Treatment conservative (steroid therapy, radiation, curettage)
      www.eyst.org
    • Langerhans Cell Histiocytosis www.eyst.org
    • Osteoid osteoma
      • 2 nd and 3 rd decades of life
      • Any bone, but femur and tibia commonly
      • cortical or cancellous
      • Typically pain worse at night relieved by aspirin or NSAID.
      • Radiologically lesion consists of a small (< 1.5 cm) central nidus with surrounding bony sclerosis
      • Treatment – medical treatment, percutaneous radiofrequency ablation, or surgical removal
      www.eyst.org
    • Osteoid osteoma www.eyst.org
    • Bone Island – Enostoses
      • Benign lesions of cancellous bone
      • Any bone
      • Asymptomatic
      • Osteopoikilosis is multiple bone islands through out skeleton
      • X rays: typically small, round or oval areas of homogeneous increased density with in cancellous bone.
      • Treated with observation and serial radiographs.
      • If painful biopsy to exclude other conditions
      www.eyst.org
    • Bone islands www.eyst.org
    • Chondroma
      • Benign lesions of hyaline cartilage.
      • Phalanges of hand are most commonly affected.
      • Usually asymptomatic rarely pathological fracture
      • Enchondromas – arise from medullary canal, Periosteal chondromas or Juxtacortical chondromas arise on surface
      • Multiple enchondromatosis – Ollier’s disease
      • Multiple enchondromatosis + Soft tissue hemangiomas = Maffuci syndrome
      • Radiographically: benign appearing tumors with intralesional calcification. Calcification is irregular and described as “strippled” “punctate” or “popcorn”. DD Chondrosarcoma
      • Treatment is observation with serial radiographs , curettage if it grows
      www.eyst.org
    • Chondroma www.eyst.org
    • Osteochondroma
      • Bony mass with form of a stalk produced by progressive endochondral ossification of growing cartilage.
      • Usually found on the metaphysis of long bones near physis.
      • Commonly distal femur, proximal tibia, and proximal humerus.
      • Clincally: pain, fracture, pressure symptoms like false aneurisms, neuropathies, palpable mass.
      • Multiple hereditary exostoses AD
      • Two types: Pedunculated or Sessile
      • Surgery(enbloc resection) is indicated when unsightly, symptomatic or any suggestion of malignancy.
      www.eyst.org
    • Osteochondroma www.eyst.org
    • Non Ossifying fibroma
      • Developmental abnormalities
      • Metaphyseal region of long bones (40% distal femur, 40% tibia, 10% fibula)
      • 2 – 20 yrs
      • Plain radiographs well lobulated lesion located eccentrically in metaphysis.
      • Asymptomatic
      www.eyst.org
    • Non Ossifying fibroma www.eyst.org
    • Cortical Desmoid
      • Is a irregularity in posteromedial aspect of distal femoral metaphysis and usually is seen in boys 10 to 15 yr old.
      www.eyst.org
    • Benign fibrous Histiocytoma
      • Diaphysis or epiphysis
      • 30 – 40 yrs
      • Radiographically it is well defined lytic, expanding lesion with little periosteal reaction
      • Extended curettage or wide resection
      www.eyst.org
    • Fibrous dysplasia
      • Developmental anomaly
      • Replacement of normal bone and marrow by fibrous tissue and small, woven spicules of bone.
      • Radiographic appearance is lucent area with lucent area having a granular, ground-glass appearance
      • Surgical treatment is indicated when there is severe deformity, pathological fracture occurs or when significant pain exists.
      • Bisphosphonates treatment is probably beneficial.
      www.eyst.org
    • Fibrous dysplasia www.eyst.org
    • Osteofibrous Dysplasia
      • Ossifying fibroma of long bones
      • Tibia and Fibula (middle third of Tibia)
      • Diaphyseal
      • Tibia is enlarged and often bowed anteriorly
      • No pain unless pathological fracture
      • Radiographs show eccentric intracortical osteolysis with expansion of cortex
      www.eyst.org
    • Osteofibrous Dysplasia www.eyst.org
    • Desmoplastic fibroma
      • Locally aggressive
      • 2 nd and 3 rd decades
      • Pain is complaint
      • Radiographs show well circumscribed lytic lesion with a narrow zone of transition and frequently a reactive bone.
      • Treatment wide resection
      www.eyst.org
    • Desomplastic fibroma www.eyst.org
    • Unicameral bone cyst
      • First 2 decades
      • M:F is 2:1
      • Common in proximal humerus and femur, in adults common in pelvis and calcaneum.
      • Asymptomatic unless pathological fracture
      • Radiographs centrally located , purely lytic lesion with well marginated outline. Fallen fragment sign
      • Small, asymptomatic lesions treated with observation and serial radiographs
      • Large, symptomatic, and lesions in lower extremity are treated with curettage or aspiration and injection.
      www.eyst.org
    • Unicameral bone cyst www.eyst.org
    • Aneurysmal bone cyst
      • Locally destructive blood filled reactive lesions of bone
      • Proximal humerus, distal femur, proximal tibia and spine
      • < 20 yrs
      • Mild to moderate pain, neurological symptoms in spinal patient.
      • Radiographically expansile lytic lesion that elevates periosteum, but remains contained by a thin shell of cortical bone.
      • Treatment with extended curettage and grafting
      www.eyst.org
    • Aneurysmal bone cyst www.eyst.org
    • Intraosseous Ganglion Cyst
      • Distal tibia, around knee and shoulder
      • Intra osseous extensions of ganglia of local soft tissues
      www.eyst.org
    • Epidermoid cyst
      • Skull
      • Radiographically they appear as rarefied defects surrounded by sclerotic bone
      www.eyst.org
    • Lipoma
      • Asymptomatic
      www.eyst.org
    • Hemangioma
      • Vertebral bodies and skull
      • Radiographically in spine it is characteristic with thickened, vertically oriented trabecuale giving the classic “jailhouse” appearance
      www.eyst.org