Bone tumours

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					Bone tumours
    Classification of bone tumours
n   Osseous
        n   Osteoma
        n   Osteoid osteoma
        n   Osteoblastoma
        n   Osteosarcoma
n   Cartilagenous lesions
        n   Osteochondroma
        n   Multiple hereditary osteochondromatosis
        n   Enchondroma
        n   Multiple enchondromatosis (Ollier’s disease)
        n   Chondroblastoma
        n   Chondromyxoid fibroma
        n   Chondrosaroma
n   Fibrous lesions
        n   Non ossifying fibroma
        n   Fibrous dysplasia
        n   Fibrosarcoma
        n   Malignant fibrous histiocytoma
n   Small (round) cell sarcoma
        n   Ewing’s sarcoma
        n   Neuroectodermal tumour of bone
n   Giant cell lesions
        n   Giant cell tumours
n   Vascular lesions
n   Chordoma
n   Lesions of hematopoeitic, lymphoid and histiocytic
    lesions
        n   Myeloma
        n   Lymphoma
n   Exostosis (osteochondroma)
n   Osteoid osteoma
n   Osteoclastoma
n   Osteosarcoma
n   Ewing’s tumour
General approach to muscloskeletal
             tumours
n   Presentation of muscloskeletal tumour may be
    n Pain
    n Mass

    n Abnormal radiographic finding during evaluation of
      unrelated problem
n   Pain of a muscloskeletal tumour
    n   Initially may be activity related, but the patient with a
        malignancy of bone often complains of progressive pain at
        rest and at night.
    n   Patients with benign bone tumors also may have activity-
        related pain if the lesion is large enough to weaken the bone.
    n   Other benign lesions, most notably osteoid osteoma, may
        initially cause night pain.
    n   Conversely, patients with soft tissue tumors rarely complain
        of pain but more often complain of a mass. Exceptions to
        this rule are patients with nerve sheath tumors who have pain
        or neurological signs
                     Incidence
n   Age … the most important information
    obtained from history. Muscloskelet neoplasms
    occur within specific age ranges
n   Sex …
n   Race … Ewing’s sarcoma is rare in African race
n   Family history..
    n Multiple hereditary exostosis
    n Neurofibromatosis
          Physical examination
n   Mass
n   Atrophy of surrounding musculature
n   Neurological examination
n   Distal pulse
n   Skin lesion … Café au lait patches
n   LN … sarcomas are rarely give LN metastases
        Radiographic Investigations
n   Radiographic
    n   Plane x-rays … the gold standard in bone tumours
    n   C.T.
    n   Bone scan
    n   MRI
    n   Ultrasonography
    n   Angiography
    n   Gallium scans
    n   PET
             Radiographic evaluation
n   Site of the lesion
     n   Bone affected
     n   Segment of bone … metaphyseal, epiphyseal or diaphyseal
     n   Position in the transverse plane … central, eccentric, cortical,
         juxtacortical, periosteal or soft tissue
n   Rate of growth
     n   Pattern of bone destruction
     n   Margin
n   Visible tumour matrix
     n   Tumour matrix
     n   Any calcificatoin
n   Internal or external trabeculae
n   Cortical pentration, erosion or expansion
n   Periosteal response
n   Soft tissue mass
n   Rest of the bone
n   1 – Ewing’s sarcoma
n   3 – osteoid osteoma
n   8 – Exostosis
n   9 – Ostesarcoma
n   11 - Osteoclastoma
n   Patterns of bone destruction
    n   Geographical
    n   Moth eaten
    n   Permeative
n   Periosteal reaction
    n Codman’s triangle
    n Onion peel… Ewing’s

    n Sun burst … osteosarcoma
n   Visible matrix
    n Mineralized osteoid
      may be solid, cloud
      like or ivory like
    n Mineralized
      chondroid may be
      stippled, flocculent,
      or rings and arcs
             Lab Investigations
n   Lab
    n CBC … infections and leukemia
    n ESR … infection, Ewing’s, leukemia, lymphoma
    n S. protein electrophoresis … multiple myeloma
    n PSA … prostatic tumours
    n S. Ca … metastatic disease, multiple myeloma,
      hyperparathyroidism.
    n Blood urea and creatinine …
    n S. Alkaline phosphatase and urinary pyridinium cross
      links
    n PTH
Biopsy
Basic Work Up
        Exostosis (osteochondroma)
n   Commonest benign tumour … 40% of benign tumours
n   Age: Adolescents and young adults
     n Site: Metaphysis of any bone formed in cartilage
    n   1/3 around knee
n   Pathology:
v   Gross : sessile or pedunculated mass
              consist of
v   Microscopic:
v   Origin :the lesion may develop from abnormal part of epiphyseal
    cartilage
n   X-ray
n   Complications
    n   Fracture stalk
    n   Sarcomatous change (chondrosarcoma)
    n   Bursitis
    n   Compression on neurovascular structures

    n   Treatment
Exostosis (osteochondroma)
Multiple hereditary exostosis
                       Osteoid osteoma
n   Age : Children and adolescents … 70% <20 years old
n   Site: Long bones particularly shaft of femur and tibia
n   X-ray
     n   Sharply rounded or ovoid radiolucent area in one side of cortex
     n   Less than 2 cm
     n   Surrounded by dense sclerosis
     n   Small dense shadow of calcified osteoid (nidus) may be present
n   Bone scan … double density sign
n   C.T. is often required to localize the lesion accurately. CT may detect the
    nidus, whereas roentgenograms show only sclerosis.
n   Prognosis
     n   Course is limited
     n   After few years, osteoid tissue becomes completely calcified, pain disappear and
         radiolucent area become filled with bone
n   Treatment
     n   block resection of the nidus
     n   shave the reactive bone with a sharp osteotome until the nidus is encountered,
         then curet the exposed nidus
     n   outpatient percutaneous radiofrequency ablation
                       Osteoclastoma
n   Age: 20-40 years. Diagnosis prior to skeletal maturity is suspect
n   Site:
    n   75% of GCT occur in ends of long bones and involve epiphysis. The
        hallmark of the tumour is extension into subchondral bone in close
        proximity to articular cartilage (perforation of cartilage may occur)
    n   75% of GCT occurs in ends of long bones, 75% of which is around knee.
        Distal end of radius is the next common site. Proximal end of humerus,
        phalanges, talus and mandible.
n   X-ray
    n   End of long bones is occupied by an osteolytic lesion which may reach
        articular surface
    n   Cortex is thinned and may be expanded or fractured
    n   Lesion is well defined with no extension into medullary cavity
    n   Metaphyseal extension may be marginated but is not sclerotic
    n   The outline of the lesion resembles “Soap bubble appearance”
    n   NO
n   Pathology
n   Treatment
                          Osteosarcoma
n   Age:
     n   10-25 years
     n   When it occur after 40 years, is usually due to …
          n   Malignant change in Paget’s disease
          n   Irradiated bone
          n   Polycystic fibrous dysplasia
          n   Multiple exo
n   Site:
    Distal end of femur, proximal end of tibia, proximal end of
    humerus are commonest
n   X-ray
     n   Metaphyseal bone destruction with new bone formation
         together with permeative growth pattern, cortical destruction,
         sunrise periosteal reaction and Codman’s triangle
     n   Soft tissue shadow
                          Pathology
n   Gross picture:
v   Fusiform swelling that extend along medulla and invade cortex
v   Consistency variable according to predominant histological
    composition
        sclerosing tumour :fibrotic elements & new bone formation
        Osteolytic tumour : area of hemorrhage & cyst formation
v   May be highly vascular “Telangiectatic “
v   Lobulated cartilaginous appearance
n   Microscopic picture :
            Malignant Stroma:
ü   Spindle shaped cells that show pleomorphism, hyperchromatism and mulitple mitotic
    figures
ü   Giant cells


                Matrix:
q   Osteoid
q   Fields of cartilage and fibrous tissue
q   Area of hemorrhage and necrosis
                      Classification
q Location        of tumour within bone :
I.   intramedullary “Convential “
II. Intracortical

III. Periosteal

IV. Parosteal



q      Cellular differention:
1)   High grade
2)   Low grade
q Histological         composition:
1.   Osteoblastic
2.   Chondroblastic
3.   Fibroblastic
4.   Telangiectatic
5.   Small cell


q    Number of foci involvement:
v    Single
v    Multicenteric


q Status      of underlying bone
ü    Normal
ü    Site of disease
n   Subtypes:
o    Convential 85%
o   Telangiectatic
o   Low grade intramedullary
o   Small cell osteosacoma
o   Intracortical
o   Parosteal
o   Periosteal
o   High grade surface O.S
o   Extra osseous
                 Treatment
n   Neoadjuvant chemotherapy
n   Wide or radical surgery
n   Adjuvant chemotherapy
                       Ewing’s sarcoma
n   Second most common bone sarcoma of childhood. It’s approximately ½ as
    frequent as OS
n   Age: 5-20 years, rare <5 years
n   Site:
     n   Pedeliction for long tubular bones. Femur is the single most common site
         (Ewing’s is said to be typically diaphyseal but in several large series metaphyseal
         lesions dominate)
     n   Pelvis and ribs are the most common flat bone sites
n   Clinical picture … may mimic acute osteomyelitis
n   X-ray
     n   Permeative or moth – eaten areas of central destruction occurring especially in
         mid shaft of long bones without evidence of new bone formation
     n   Lamellar periosteal reaction of “ onion – peel type ”or sun – burst appearance is
         seen
n   MRI … MRI also is useful to evaluate the extent of the soft tissue mass,
    which often is very large
n   Patthology
n   Treatment
    n   neoadjuvant or adjuvant chemotherapy, or both, to treat distant metastases that
        may or may not be readily apparent at the initial staging
    n   Local treatment of the primary lesion is more controversial. Ewing sarcoma is
        very radiosensitive, large, central, unresectable tumors often are treated with
        radiation
Simple bone cyst
                                              ABC
n   The aneurysmal bone cyst is an unusual entity which is most likely the result of trauma or a tumor
    induced process. In approximately one third of cases, the pre-existing lesion can be identified.
    The most common of these pre-existing lesions are giant cell tumors which account for
    approximately one fourth of aneurysmal bone cysts. Other possible underlying etiologies include
    osteoblastomas, angiomas, and chondroblastomas. The vast majority of patients present with pain
    and/or swelling. Patients are most typically under the age of 20 with the vast majority of
    aneurysmal bone cysts presenting in patients under the age of 30. Greater than 50% of
    aneurysmal bone cysts occur in long bones with approximately 12 to 30% occurring in the spine.
    Aneurysmal bone cysts of the flat bones are uncommon, of which about 50% occur in the pelvis.
n   Radiographic findings include an eccentric, lytic lesion, with an expanded, remodeled bony
    contour. Radiographs on occasion may show internal densities characteristic of chondroid matrix.
    In addition, internal septations are common. CT scanning is helpful in defining the lesion,
    especially in areas in which the bony anatomy is complex. MR imaging will generally demonstrate
    a well-defined, lobulated lesion with internal septations. In addition, multiple fluid fluid levels will
    be identified on MRI. The appropriate treatment of an aneurysmal bone cyst include identifying
    its specific pre-existing lesion. Treatment is generally directed at surgical removal of the entire
    lesion versus curettage with bone grafting.
Ankylosing spondylitis

				
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