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Bone Tumors
Pavel Douša
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• The essential prerequisite for effective diagnosis and treatment of patients with bone tumors is an interdisciplinary cooperation.
• On the treatment of these patients taka part pediatricians, general practitioners, orthopedic surgeons, pathologists, oncologists, radiologists and rehabilitation specialists.
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Examination• Medical history• Clinical examination• X-ray• Laboratory exams• CT, MRI, angiography• Biopsy
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Painful tumors - clinical features associated with bone tumors include pain,
deformity and impairment of function; - pain is feature common to almost all malignant tumors of bone,
but may or may not be present in benign lesions; - malignant bone tumors are usually associated with deep, aching pain,
which, although not sharp and sometimes not severe, is quite distressing to the patient because of its constancy;
- pain that persists at night & is not relieved by rest is suggestive of malignancy;however, certain benign tumors, such as osteoid osteoma, are characterized by nocturnal pain;
- mild, dull, aching back pain that suddenly becomes excruciating w/ such insignificant trauma as sitting down a bit hard suggests Pathologic compression frx in vertebra involved by metastatic cancer;
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Pathologic fractures
- many tumors gradually erode bone and weaken it to the extent that it cannot withstand the strain of ordinary use;
- in such situations, minimal trauma may precipitate pathologic frx;- if there were no symptoms prior to time of frx, chances are that
tumor is benign;- solitary bone cyst, fibrous dysplasia, nonossifying fibroma,
and enchondromas are benign lesions that frequently are first noted because of fracture;
- on other hand, if frx has been preceded by dull, aching pain, possibility of malignant primary bone tumor or metastasis is likely;
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Benign Bone Tumors
- benign bone tumors generally do not extend beyond the cortex of bone;
- often these tumors are surrounded by a radiodense margin of bone;- where as metaphyseal tumors are common, diaphyseal tumors are uncommon;- diff dx of benign diaphyseal tumors includes EOG and fibrous dysplasia
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Radiographic Features of Bone Tumors
- note whether the bone formed in tumor is being produced by actual tumor cells or by normal osteoblasts reacting to tumor
- while several benign tumors may form bone, only osteogenic sarcoma is a malignant bone forming tumor- host bone often responds to a tumor by combination of resorption and bone formation- reactive bone that forms the thin sclerotic border of slowly growing tumor may be mature lamellar bone- some tumors, such as typical osteochondroma, are so characteristic that x-rays alone can establish the diagnosis
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Malignant lesions - are suggested if the boundaries of the tumor are ill-defined,
if there are no sharp borders, if the lesion has a mottled appearance, and if it has broken out of the confines of the bone and destroyed cortex, malignancy is to be suspected
- malignant tumor cells that extend through the cortex may elevate periosteum and stimulate it to produce a small triangle of reactive bone (Codman's triangle) where periosteum is lifted from the shaft;
- formation of new osseous tissue outside involved bone is suggestive of malignancy but can also be found in cases of infection and in myositis ossificans;
- subperiosteal new bone formation, which has laminated or onionskin appearance, is seen in Ewing's sarcoma, but it may also be found in other conditions, such as infection;
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Biopsy of MSK tumors May accomplished with a large needle or by open surgical
methods; - needle bx - fine needle biopsy: - relies on cytologal interpretation
- its accuracy ranges between 65 to 95% - core needle biopsy:
- uses trocar cannula system, - allows for immuno-histochemical analysis;
- accuracy ranges between 75 to 95%; - incisional open bx: - where several areas of a tumor must be evaluated
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Surgical Staging System for Musculoskeletal Tumors
Stage determined by three different sub-categories
-Grade: Histology with aid of radiographic findings and clinical correlationG 0: benign grade, uniform cell type without atypia, no mitosesG 1: Low grade, uniform cell type without atypia, few mitosesG 2: High grade, atypical nuclei, mitoses pronounced
-Site: T 1: Intracompartmental (Confined within limits of periosteum)T 2: Extracompartmental (Breach in an adjacent joint cartilage, bone cortex (or periosteum), fascia lata, quadriceps, and joint capsule)
-Metastasis: M 0: No identifiable skip lesions or distant metastases.M 1: Any skip lesions, regional lymph nodes, or distant metastases.
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Surgical Staging System
Benign: 1 Latent G 0 T 0 M 0
2 Active G 0 T 0 M 0
3 Aggressive G 0 T 1-2 M 0-1
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Enneking's Staging System of Malignant Bone Tumors
Ia G 1 T 1 M 0Ib G 1 T 2 M 0IIa G 2 T 1 M 0IIb G 2 T 2 M 0IIIa G 1-2 T 1 M 1IIIb G 1-2 T 2 M 1
Treatment of any potentially malignant bone tumor always begins with staging. Chest CT scan to evaluate for pulmonary metastasis, bone scan orskeletal survey for distant metastases or skip lesions.
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Division of Bone TumorsOsseous Osteoid osteoma Classic osteosarcoma Osteoblastoma OsteochondromaCartilaginous Enchondroma Chondrosarcoma Chondroblastoma OsteochondromaFibrous Fibrous Dysplasia Malignant fibrous histiocytoma Ossifying fibroma
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Division of Bone Tumors
Reticuloendothelial Eosinophilic granuloma Ewing's sarcomaVascular Aneurysmal bone cyst Angiosarcoma HemangiomaUnknown origin Simple bone cyst Giant cell sarcoma Giant Cell Tumor ChordomaMisc Multiple Myeloma
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Treatment
• Surgical treatment
• Radiotherapy
• Chemotherapy
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Osteochondroma / Osteocartilaginous Exostosis
- a developmental dysplasia of peripheral growth plate which forms a cartilage capped projection of bone found near metaphyses of long bones- peripheral chondroblast grows outward from the metaphysis, acting as an ectopic growth plate
- it is the most common benign bone tumor - usually occurs in long bones, but may occur any
bone that is preformed in cartilage
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Treatment
- surgical resection is indicated for persistant irritation (from bursitis) or for neurovascular comprimise; - surgical resection is also indicated for continued osteochondroma growth after skeletal maturity (in which case malignancy is suspected); - definitive treatment includes marginal excision of an active exostosis,
- prognosis for a solitary exostosis is excellent (< 5% recurrence following marginal excision);
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Osteoid Osteoma
- benign osseous tumor usually less than 1.5 cm in diameter, comprising 11% of all benign bone tumors, no cases of malignant transformation has been reported
- occurs primarily in adolescents and less often in children; - presenting symptom is well localized pain which may be
more severe at night & is relieved by aspirin or other prostaglandin inhibitors
- location: - most common site is proximal femur & diaphysis of long
bones; less often: foot (talus, navicular, or calcaneus) & in posterior spine
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Osteoid Osteoma
Radiographs: - intense bony rxn to small nidus is hallmark of osteoid osteoma, however, may be difficult to see on x-rays;CT Scan: - nidus is best localized w/ CT - tell radiologist of diff dx:
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Treatment:
- osteoid osteoma may resolve spontaneously w/ time (especially when located in the hand), however, most patients prefer not to wait 2 to 4 years for resolution; - when nidus is located in a low stress area such as metaphysis, treatment should consist of en bloc excision w/ surrounding small block of reactive bone;
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Osteosarcoma
- malignant tumor of bone in which neoplastic osteoid is produced by a proliferating spindle cell stroma;
- most common primary malignant bone tumor of mesenchymal derivation;
- arises in adolescents & affects males slightly more often than females.
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Osteosarcoma
affected sites: - occurs in region of knee (distal femur or
proximal tibia) in 50% of pts; - other sites include proximal humerus,
proximal femur, & pelvis; - most osteosarcomas occur in the metaphysis; - infrequently occurs in the spine
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Osteosarcoma
Classification: - classic - telangiectatic - parosteal - periosteal________________________________________Clinical Presentation: - initial symtom is a painful bony mass; - significant number of osteosarcomas in adults
occur in assoc w/ Pagets disease;
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Treatment of Classic Osteosarcoma - surgery alone as a treatment yields only a 20% survival rate
- non metastatic disease treated with chemotherapy and surgery, expected survival is 60-80% - chemotherapy (neoadjuvant, adjuvant): - with adjuvant chemotherapy, the 5 year survival rate is > 50%; - some surgeons insist on preoperative chemotherapy in the management of osteosarcoma; - good response to chemotherapy is indicated by 90% tumor necrosis on biopsy;
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Enchondroma
benign and asymptomatic cartilaginous tumor of bone which most often occurs in adolescents or young adults; - it is an intramedullary cartilage lesion located in central metaphysis;
- most often affects small tubular bones of hands or feet (40-65%);
- may also affect proximal humerus and femoral shaft;
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Treatment
- asymptomatic solitary enchondromas may be followed non operatively w/ serial radiographs; - prognosis for benign enchondroma is excellent. - if solitary or multiple enchondromas become symptomatic or begin to enlarge, they may require biopsy to r/o malignancy;
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Ewing's Sarcoma
- prevalence: - it represents approx 7% of all primary bone malignancies; - Ewing's sarcoma is more common in males
- source: - uncertain origin; - may arise from primitive reticulum cells of marrow;
- age: - it is a tumor of children & young adults, w/ most cases occurring between ages of 10 and 20 years; - 3% occurs in children less than 3 years of age; - 64% of Ewing's sarcoma occur in the second decade
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Clinical Presentation - may mimic osteomyelitis w/ fever, elevated WBC, & sed rate, wt
loss, anemia; - sites:
- femoral diaphysis > pelvis, tibia, humerus, fibula & ribs; - develops in axial skeleton in large proportion of pts (about 40 %); - tumors in pelvis are typically detected later & are therefore larger, with a poorer prognosi
- distant metastases are noted at diagnosis in about 20% of cases;
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Prognosis
- potentially the most lethal of all the bone tumors;- most important adverse prognostic factor was metastatic disease detectable at the time of diagnosis;- it almost always presents as a stage IIb lesion;- before routine use of adjuvant chemo, pts w/ Ewing's were treated w/ XRT alone, & 5 year survival rate was no more than 15%;- w/ use of adjuvant chemo, 5 year survival rate to more than 60%;
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Treatment
treatment consists of chemotherapy, surgical resection & possible XRT;
- radiotherapy: - approx 75-85 % of primary ewing's tumors are sensitive - obviously XRT is not desirable in children because of its
effect on the growing limb and because of the possibility for causing secondary tumors (can be up to 20%);
- chemotherapy:- addition of chemotherapy with surgery increases survival to greater than 70% at 5 years;
- wide surgical excision:- limb salvage w/ insertion of long stem tibial component:
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Malignant Fibrous Histiocytoma
• Osseous Sarcoma: - occur less often in bone than in soft tissue; - occurs in older patient population & may arise out of Pagets Dz or from a bone infarct - it usually presents as an aggressive stage IIb sarcoma; - pathologic frx may be the presenting feature; - has a high rate of metastasis, especially to regional lymph nodes.
• Clinical Presentation: - presents in adults; - may present as large mass involving deep soft tissues of proximal limbs; - may present as a small, superficial, low grade tumor in the distal limbs;
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Treatment
- attention is directed to ruling out metastases to the regional lymph nodes; - low grade stage I tumors usually develop in more peripheral & superfical locations than do high grade tumors. - treatment w/ wide surgical excision is usually adequate, w/ amputation reserved for multiple recurrances. - stage II tumors require excision w/ wide margins or amputation; - preop XRT or postoperative chemo, or both, may be indicated;
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Simple Bone Cyst - a membrane lined cavity containing a clear yellow
fluid.- occurrs most often in children 4-10 years of age.- lesions remain asymptomatic unless complicated by
fracture.- they enlarge during skeletal growth and become
inactive, or latent, after skeletal maturity.- lesion appears to arise from the growth plate & in
early stages, lesion is lies adjacent to growth plate.- predilection for the metaphysis of long bones;
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Treatment- goal of treatment is prevention of pathologic fracture;- simple cysts are treated with curettage and bone
grafting;- recurrence is high for active cysts (50%) & low for
latent cysts (10%);- alternative treatments:
- steroids - 80-200 mg of methylprednisolone infused into cavity; - autologous bone marrow injection: - multiple drill holes
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Multiple Myeloma
- myeloma is malignant tumor of plasma cells arising from a single clone;
- multiple myeloma accounts for > 40% of primary malignant tumors of bone.
- it is most common malignant primary tumor of bone- may arise as single intraosseous tumor but more often it
develops as multiple painful lesions throughout skeleton (multiple myeloma);
- pts present in their middle fifties or older (60-70 yr)- constitutional symptoms, anemia, thrombocytopenia, and
renal failure;
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Treatment
- XRT: - myeloma is sensitive to XRT, & reossification of tumor defects may ocurr within several months. - XRT is recommended for intractable bone pain, esp if pain is localized; - it can be dramatically effective in relieving symptoms;
- Chemo: - when dz is disseminated, chemo is indicated
- 5 year survival remains under 30%; - Surgical Fixation: